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href="#Cofactor_for_tyrosine_hydroxylase"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.3</span> <span>Cofactor for tyrosine hydroxylase</span> </div> </a> <ul id="toc-Cofactor_for_tyrosine_hydroxylase-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Cofactor_for_nitric_oxide_synthase" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Cofactor_for_nitric_oxide_synthase"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.4</span> <span>Cofactor for nitric oxide synthase</span> </div> </a> <ul id="toc-Cofactor_for_nitric_oxide_synthase-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Cofactor_for_ether_lipid_oxidase" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Cofactor_for_ether_lipid_oxidase"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.5</span> <span>Cofactor for ether lipid oxidase</span> </div> </a> <ul id="toc-Cofactor_for_ether_lipid_oxidase-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-History" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#History"> <div class="vector-toc-text"> <span class="vector-toc-numb">5</span> <span>History</span> </div> </a> <ul id="toc-History-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Biosynthesis_and_recycling" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Biosynthesis_and_recycling"> <div class="vector-toc-text"> <span class="vector-toc-numb">6</span> <span>Biosynthesis and recycling</span> </div> </a> <ul id="toc-Biosynthesis_and_recycling-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Research" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Research"> <div class="vector-toc-text"> <span class="vector-toc-numb">7</span> <span>Research</span> </div> </a> <button aria-controls="toc-Research-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Research subsection</span> </button> <ul id="toc-Research-sublist" class="vector-toc-list"> <li id="toc-Depression" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Depression"> <div class="vector-toc-text"> <span class="vector-toc-numb">7.1</span> <span>Depression</span> </div> </a> <ul id="toc-Depression-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Autism" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Autism"> <div class="vector-toc-text"> <span class="vector-toc-numb">7.2</span> <span>Autism</span> </div> </a> <ul id="toc-Autism-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Cardiovascular_disease" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Cardiovascular_disease"> <div class="vector-toc-text"> <span class="vector-toc-numb">7.3</span> <span>Cardiovascular disease</span> </div> </a> <ul id="toc-Cardiovascular_disease-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Neuroprotection_in_prenatal_hypoxia" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Neuroprotection_in_prenatal_hypoxia"> <div class="vector-toc-text"> <span class="vector-toc-numb">7.4</span> <span>Neuroprotection in prenatal hypoxia</span> </div> </a> <ul id="toc-Neuroprotection_in_prenatal_hypoxia-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Programmed_cell_death" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Programmed_cell_death"> <div class="vector-toc-text"> <span class="vector-toc-numb">7.5</span> <span>Programmed cell death</span> </div> </a> <ul id="toc-Programmed_cell_death-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-References" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#References"> <div class="vector-toc-text"> <span class="vector-toc-numb">8</span> <span>References</span> </div> </a> <ul id="toc-References-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Further_reading" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Further_reading"> <div class="vector-toc-text"> <span class="vector-toc-numb">9</span> <span>Further reading</span> </div> </a> <ul id="toc-Further_reading-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-External_links" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#External_links"> <div class="vector-toc-text"> <span class="vector-toc-numb">10</span> <span>External 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Available in 20 languages" > <label id="p-lang-btn-label" for="p-lang-btn-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--action-progressive mw-portlet-lang-heading-20" aria-hidden="true" ><span class="vector-icon mw-ui-icon-language-progressive mw-ui-icon-wikimedia-language-progressive"></span> <span class="vector-dropdown-label-text">20 languages</span> </label> <div class="vector-dropdown-content"> <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li class="interlanguage-link interwiki-ar mw-list-item"><a href="https://ar.wikipedia.org/wiki/%D8%B1%D8%A8%D8%A7%D8%B9%D9%8A_%D9%87%D9%8A%D8%AF%D8%B1%D9%88_%D8%A7%D9%84%D8%A8%D9%8A%D9%88%D8%A8%D8%AA%D9%8A%D8%B1%D9%8A%D9%86" title="رباعي هيدرو البيوبتيرين – Arabic" lang="ar" hreflang="ar" data-title="رباعي هيدرو البيوبتيرين" data-language-autonym="العربية" data-language-local-name="Arabic" class="interlanguage-link-target"><span>العربية</span></a></li><li class="interlanguage-link interwiki-azb mw-list-item"><a href="https://azb.wikipedia.org/wiki/%D8%AA%D8%AA%D8%B1%D8%A7%D9%87%DB%8C%D8%AF%D8%B1%D9%88_%D8%A8%DB%8C%D9%88%D9%BE%D8%AA%D8%B1%DB%8C%D9%86" title="تتراهیدرو بیوپترین – South Azerbaijani" lang="azb" hreflang="azb" data-title="تتراهیدرو بیوپترین" data-language-autonym="تۆرکجه" data-language-local-name="South Azerbaijani" class="interlanguage-link-target"><span>تۆرکجه</span></a></li><li class="interlanguage-link interwiki-de mw-list-item"><a href="https://de.wikipedia.org/wiki/Sapropterin" title="Sapropterin – German" lang="de" hreflang="de" data-title="Sapropterin" data-language-autonym="Deutsch" data-language-local-name="German" class="interlanguage-link-target"><span>Deutsch</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/Tetrahidrobiopterina" title="Tetrahidrobiopterina – Spanish" lang="es" hreflang="es" data-title="Tetrahidrobiopterina" data-language-autonym="Español" data-language-local-name="Spanish" class="interlanguage-link-target"><span>Español</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/T%C3%A9trahydrobiopt%C3%A9rine" title="Tétrahydrobioptérine – French" lang="fr" hreflang="fr" data-title="Tétrahydrobioptérine" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-ko mw-list-item"><a href="https://ko.wikipedia.org/wiki/%ED%85%8C%ED%8A%B8%EB%9D%BC%ED%95%98%EC%9D%B4%EB%93%9C%EB%A1%9C%EB%B9%84%EC%98%A4%ED%94%84%ED%85%8C%EB%A6%B0" title="테트라하이드로비오프테린 – Korean" lang="ko" hreflang="ko" data-title="테트라하이드로비오프테린" data-language-autonym="한국어" data-language-local-name="Korean" class="interlanguage-link-target"><span>한국어</span></a></li><li class="interlanguage-link interwiki-it mw-list-item"><a href="https://it.wikipedia.org/wiki/Tetraidrobiopterina" title="Tetraidrobiopterina – Italian" lang="it" hreflang="it" data-title="Tetraidrobiopterina" data-language-autonym="Italiano" data-language-local-name="Italian" class="interlanguage-link-target"><span>Italiano</span></a></li><li class="interlanguage-link interwiki-he mw-list-item"><a href="https://he.wikipedia.org/wiki/%D7%98%D7%98%D7%A8%D7%94-%D7%94%D7%99%D7%93%D7%A8%D7%95-%D7%91%D7%99%D7%95%D7%A4%D7%98%D7%A8%D7%99%D7%9F" title="טטרה-הידרו-ביופטרין – Hebrew" lang="he" hreflang="he" data-title="טטרה-הידרו-ביופטרין" data-language-autonym="עברית" data-language-local-name="Hebrew" class="interlanguage-link-target"><span>עברית</span></a></li><li class="interlanguage-link interwiki-ms mw-list-item"><a href="https://ms.wikipedia.org/wiki/Tetrahidrobiopterin" title="Tetrahidrobiopterin – Malay" lang="ms" hreflang="ms" data-title="Tetrahidrobiopterin" data-language-autonym="Bahasa Melayu" data-language-local-name="Malay" class="interlanguage-link-target"><span>Bahasa Melayu</span></a></li><li class="interlanguage-link interwiki-ja mw-list-item"><a href="https://ja.wikipedia.org/wiki/%E3%83%86%E3%83%88%E3%83%A9%E3%83%92%E3%83%89%E3%83%AD%E3%83%93%E3%82%AA%E3%83%97%E3%83%86%E3%83%AA%E3%83%B3" title="テトラヒドロビオプテリン – Japanese" lang="ja" hreflang="ja" data-title="テトラヒドロビオプテリン" data-language-autonym="日本語" data-language-local-name="Japanese" class="interlanguage-link-target"><span>日本語</span></a></li><li class="interlanguage-link interwiki-or mw-list-item"><a href="https://or.wikipedia.org/wiki/%E0%AC%B8%E0%AC%BE%E0%AC%AA%E0%AD%8D%E0%AC%B0%E0%AD%8B%E0%AC%AA%E0%AD%8D%E0%AC%9F%E0%AD%87%E0%AC%B0%E0%AC%BF%E0%AC%A8" title="ସାପ୍ରୋପ୍ଟେରିନ – Odia" lang="or" hreflang="or" data-title="ସାପ୍ରୋପ୍ଟେରିନ" data-language-autonym="ଓଡ଼ିଆ" data-language-local-name="Odia" class="interlanguage-link-target"><span>ଓଡ଼ିଆ</span></a></li><li class="interlanguage-link interwiki-uz mw-list-item"><a href="https://uz.wikipedia.org/wiki/Tetragidrobiopterin" title="Tetragidrobiopterin – Uzbek" lang="uz" hreflang="uz" data-title="Tetragidrobiopterin" data-language-autonym="Oʻzbekcha / ўзбекча" data-language-local-name="Uzbek" class="interlanguage-link-target"><span>Oʻzbekcha / ўзбекча</span></a></li><li class="interlanguage-link interwiki-pl mw-list-item"><a href="https://pl.wikipedia.org/wiki/Sapropteryna" title="Sapropteryna – Polish" lang="pl" hreflang="pl" data-title="Sapropteryna" data-language-autonym="Polski" data-language-local-name="Polish" class="interlanguage-link-target"><span>Polski</span></a></li><li class="interlanguage-link interwiki-pt mw-list-item"><a href="https://pt.wikipedia.org/wiki/Tetraidrobiopterina" title="Tetraidrobiopterina – Portuguese" lang="pt" hreflang="pt" data-title="Tetraidrobiopterina" data-language-autonym="Português" data-language-local-name="Portuguese" class="interlanguage-link-target"><span>Português</span></a></li><li class="interlanguage-link interwiki-ru mw-list-item"><a href="https://ru.wikipedia.org/wiki/%D0%A2%D0%B5%D1%82%D1%80%D0%B0%D0%B3%D0%B8%D0%B4%D1%80%D0%BE%D0%B1%D0%B8%D0%BE%D0%BF%D1%82%D0%B5%D1%80%D0%B8%D0%BD" title="Тетрагидробиоптерин – Russian" lang="ru" hreflang="ru" data-title="Тетрагидробиоптерин" data-language-autonym="Русский" data-language-local-name="Russian" class="interlanguage-link-target"><span>Русский</span></a></li><li class="interlanguage-link interwiki-sk mw-list-item"><a href="https://sk.wikipedia.org/wiki/Tetrahydrobiopter%C3%ADn" title="Tetrahydrobiopterín – Slovak" lang="sk" hreflang="sk" data-title="Tetrahydrobiopterín" data-language-autonym="Slovenčina" data-language-local-name="Slovak" class="interlanguage-link-target"><span>Slovenčina</span></a></li><li class="interlanguage-link interwiki-sr mw-list-item"><a href="https://sr.wikipedia.org/wiki/Tetrahidrobiopterin" title="Tetrahidrobiopterin – Serbian" lang="sr" hreflang="sr" data-title="Tetrahidrobiopterin" data-language-autonym="Српски / srpski" data-language-local-name="Serbian" class="interlanguage-link-target"><span>Српски / srpski</span></a></li><li class="interlanguage-link interwiki-sh mw-list-item"><a href="https://sh.wikipedia.org/wiki/Tetrahidrobiopterin" title="Tetrahidrobiopterin – Serbo-Croatian" lang="sh" hreflang="sh" data-title="Tetrahidrobiopterin" data-language-autonym="Srpskohrvatski / српскохрватски" data-language-local-name="Serbo-Croatian" class="interlanguage-link-target"><span>Srpskohrvatski / српскохрватски</span></a></li><li class="interlanguage-link interwiki-fi mw-list-item"><a href="https://fi.wikipedia.org/wiki/Tetrahydrobiopteriini" title="Tetrahydrobiopteriini – Finnish" lang="fi" hreflang="fi" data-title="Tetrahydrobiopteriini" data-language-autonym="Suomi" data-language-local-name="Finnish" class="interlanguage-link-target"><span>Suomi</span></a></li><li class="interlanguage-link interwiki-zh mw-list-item"><a href="https://zh.wikipedia.org/wiki/%E5%9B%9B%E6%B0%A2%E7%94%9F%E7%89%A9%E8%9D%B6%E5%91%A4" title="四氢生物蝶呤 – Chinese" lang="zh" hreflang="zh" data-title="四氢生物蝶呤" data-language-autonym="中文" data-language-local-name="Chinese" class="interlanguage-link-target"><span>中文</span></a></li> </ul> <div class="after-portlet after-portlet-lang"><span class="wb-langlinks-edit wb-langlinks-link"><a href="https://www.wikidata.org/wiki/Special:EntityPage/Q419808#sitelinks-wikipedia" title="Edit interlanguage links" class="wbc-editpage">Edit links</a></span></div> </div> </div> </div> </header> <div class="vector-page-toolbar"> <div class="vector-page-toolbar-container"> <div id="left-navigation"> <nav aria-label="Namespaces"> <div id="p-associated-pages" class="vector-menu vector-menu-tabs mw-portlet mw-portlet-associated-pages" > <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li id="ca-nstab-main" class="selected vector-tab-noicon mw-list-item"><a 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.mw-parser-output .hatnote{display:none!important}}</style><div role="note" class="hatnote navigation-not-searchable">"BH4" redirects here. For the anion, see <a href="/wiki/Borohydride" title="Borohydride">Borohydride</a>. For the edition of the Hebrew Bible, see <a href="/wiki/Biblia_Hebraica_Stuttgartensia" title="Biblia Hebraica Stuttgartensia">Biblia Hebraica Stuttgartensia</a>.</div> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Pharmaceutical compound</div> <style data-mw-deduplicate="TemplateStyles:r1269284339">@media screen{html.skin-theme-clientpref-night .mw-parser-output .dark_mode_safe img{background-color:var(--background-color-inverted,#f8f9fa)}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .dark_mode_safe img{background-color:var(--background-color-inverted,#f8f9fa)}}</style> <style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox" style="border-spacing:2px;"><caption class="infobox-title">Tetrahydrobiopterin<br /><span style="font-size:85%; font-weight:normal;"><abbr title="International nonproprietary name">INN</abbr>:</span> Sapropterin</caption><tbody><tr><td colspan="2" class="infobox-image"><span class="mw-default-size dark_mode_safe" typeof="mw:File/Frameless"><a href="/wiki/File:(6R)-Tetrahydrobiopterin_structure.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/6/63/%286R%29-Tetrahydrobiopterin_structure.svg/220px-%286R%29-Tetrahydrobiopterin_structure.svg.png" decoding="async" width="220" height="121" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/6/63/%286R%29-Tetrahydrobiopterin_structure.svg/330px-%286R%29-Tetrahydrobiopterin_structure.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/6/63/%286R%29-Tetrahydrobiopterin_structure.svg/440px-%286R%29-Tetrahydrobiopterin_structure.svg.png 2x" data-file-width="512" data-file-height="281" /></a></span></td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Clinical data</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Drug_nomenclature#Trade_names" title="Drug nomenclature">Trade names</a></th><td class="infobox-data">Kuvan, Biopten</td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;">Other names</th><td class="infobox-data">Sapropterin hydrochloride (<a href="/wiki/Japanese_Accepted_Name" title="Japanese Accepted Name">JAN</a> <small><abbr title="Japan">JP</abbr></small>), Sapropterin dihydrochloride (<a href="/wiki/United_States_Adopted_Name" title="United States Adopted Name">USAN</a> <small><abbr title="United States">US</abbr></small>)</td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/American_Society_of_Health-System_Pharmacists" title="American Society of Health-System Pharmacists">AHFS</a>/<a href="/wiki/Drugs.com" title="Drugs.com">Drugs.com</a></th><td class="infobox-data"><span title="www.drugs.com"><a rel="nofollow" class="external text" href="https://www.drugs.com/monograph/sapropterin-dihydrochloride.html">Monograph</a></span></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus</a></th><td class="infobox-data"><span title="medlineplus.gov"><a rel="nofollow" class="external text" href="https://medlineplus.gov/druginfo/meds/a608020.html">a608020</a></span></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Regulation_of_therapeutic_goods" title="Regulation of therapeutic goods">License data</a></th><td class="infobox-data"><style data-mw-deduplicate="TemplateStyles:r1126788409">.mw-parser-output .plainlist ol,.mw-parser-output .plainlist ul{line-height:inherit;list-style:none;margin:0;padding:0}.mw-parser-output .plainlist ol li,.mw-parser-output .plainlist ul li{margin-bottom:0}</style><div class="plainlist"> <ul><li><small><abbr class="country-name" title="European Union">EU</abbr></small> <a href="/wiki/European_Medicines_Agency" title="European Medicines Agency">EMA</a>: <span title="www.ema.europa.eu: 'Sapropterin'"><a rel="nofollow" class="external text" href="https://www.ema.europa.eu/en/medicines/search_api_aggregation_ema_active_substance_and_inn_common_name/Sapropterin">by INN</a></span></li> <li><small><abbr class="country-name" title="United States">US</abbr></small> <a href="/wiki/DailyMed" title="DailyMed">DailyMed</a>: <span title="dailymed.nlm.nih.gov"><a rel="nofollow" class="external text" href="https://dailymed.nlm.nih.gov/dailymed/search.cfm?labeltype=all&query=Sapropterin">Sapropterin</a></span></li> <li><small><abbr class="country-name" title="United States">US</abbr></small> <span title="www.accessdata.fda.gov"><a href="/wiki/U.S._Food_and_Drug_Administration" class="mw-redirect" title="U.S. Food and Drug Administration">FDA</a>: <a rel="nofollow" class="external text" href="https://www.accessdata.fda.gov/scripts/cder/drugsatfda/index.cfm?fuseaction=Search.SearchAction&SearchTerm=Sapropterin&SearchType=BasicSearch">Sapropterin</a></span></li></ul></div> </td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Pregnancy_category" title="Pregnancy category">Pregnancy<br />category</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"> <ul><li><small><abbr class="country-name" title="Australia">AU</abbr>:</small> B1<sup id="cite_ref-Drugs.com_pregnancy_1-0" class="reference"><a href="#cite_note-Drugs.com_pregnancy-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup></li> <li class="mw-empty-elt"></li></ul> </div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Route_of_administration" title="Route of administration">Routes of<br />administration</a></th><td class="infobox-data"><a href="/wiki/Oral_administration" title="Oral administration">By mouth</a></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Anatomical_Therapeutic_Chemical_Classification_System" title="Anatomical Therapeutic Chemical Classification System">ATC code</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><a href="/wiki/ATC_code_A16" title="ATC code A16">A16AX07</a> (<span title="www.whocc.no"><a rel="nofollow" class="external text" href="https://www.whocc.no/atc_ddd_index/?code=A16AX07">WHO</a></span>) </li></ul></div></td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Legal status</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Regulation_of_therapeutic_goods" title="Regulation of therapeutic goods">Legal status</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"> <ul><li><small><abbr class="country-name" title="Australia">AU</abbr>:</small> <a href="/wiki/Standard_for_the_Uniform_Scheduling_of_Medicines_and_Poisons#Schedule_4" title="Standard for the Uniform Scheduling of Medicines and Poisons">S4</a> (Prescription only)</li> <li><small><abbr class="country-name" title="Canada">CA</abbr></small>: <a href="/wiki/Prescription_drug" title="Prescription drug"> ℞-only</a><sup id="cite_ref-Kuvan_CA_Product_information_2-0" class="reference"><a href="#cite_note-Kuvan_CA_Product_information-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><small><abbr class="country-name" title="United States">US</abbr>:</small> <a href="/wiki/Prescription_drug" title="Prescription drug">℞-only</a><sup id="cite_ref-Kuvan_FDA_label_3-0" class="reference"><a href="#cite_note-Kuvan_FDA_label-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup></li> <li><small><abbr class="country-name" title="European Union">EU</abbr>:</small> Rx-only<sup id="cite_ref-Kuvan_EPAR_4-0" class="reference"><a href="#cite_note-Kuvan_EPAR-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li>In general: ℞ (Prescription only)</li></ul></div> </td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;"><a href="/wiki/Pharmacokinetics" title="Pharmacokinetics">Pharmacokinetic</a> data</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Biological_half-life" title="Biological half-life">Elimination <span class="nowrap">half-life</span></a></th><td class="infobox-data">4 hours (healthy adults)<br />6–7 hours (<a href="/wiki/Phenylketonuria" title="Phenylketonuria">PKU</a> patients)</td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Identifiers</th></tr><tr><td colspan="2" class="infobox-full-data"><div class="collapsible-list mw-collapsible mw-collapsed" style="text-align: left;"> <div style="line-height: 1.6em; font-weight: bold;"><div><a href="/wiki/IUPAC_nomenclature_of_chemistry" title="IUPAC nomenclature of chemistry">IUPAC name</a></div></div> <ul class="mw-collapsible-content" style="margin-top: 0; margin-bottom: 0; line-height: inherit; list-style: none; margin-left: 0; word-break:break-all; text-align:left; padding-left:1.5em; text-indent:-1.5em;"><li style="line-height: inherit; margin: 0"><div style="font-size: 97%;">(6<i>R</i>)-2-Amino-6-[(1<i>R</i>,2<i>S</i>)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydropteridin-4(1<i>H</i>)-one</div></li></ul> </div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/CAS_Registry_Number" title="CAS Registry Number">CAS Number</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="commonchemistry.cas.org"><a rel="nofollow" class="external text" href="https://commonchemistry.cas.org/detail?cas_rn=62989-33-7">62989-33-7</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li><li>as salt: <span title="commonchemistry.cas.org"><a rel="nofollow" class="external text" href="https://commonchemistry.cas.org/detail?cas_rn=69056-38-8">69056-38-8</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/PubChem#CID" title="PubChem">PubChem</a> <span style="font-weight:normal"><abbr title="Compound ID">CID</abbr></span></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="pubchem.ncbi.nlm.nih.gov"><a rel="nofollow" class="external text" href="https://pubchem.ncbi.nlm.nih.gov/compound/135398654">135398654</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Guide_to_Pharmacology" title="Guide to Pharmacology">IUPHAR/BPS</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.guidetopharmacology.org"><a rel="nofollow" class="external text" href="https://www.guidetopharmacology.org/GRAC/LigandDisplayForward?ligandId=5276">5276</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/DrugBank" title="DrugBank">DrugBank</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.drugbank.ca"><a rel="nofollow" class="external text" href="https://www.drugbank.ca/drugs/DB00360">DB00360</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li><li>as salt: <span title="www.drugbank.ca"><a rel="nofollow" class="external text" href="https://www.drugbank.ca/drugs/DBSALT001133">DBSALT001133</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ChemSpider" title="ChemSpider">ChemSpider</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.chemspider.com"><a rel="nofollow" class="external text" href="https://www.chemspider.com/Chemical-Structure.40270.html">40270</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li><li>as salt: <span title="www.chemspider.com"><a rel="nofollow" class="external text" href="https://www.chemspider.com/Chemical-Structure.552166.html">552166</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Unique_Ingredient_Identifier" title="Unique Ingredient Identifier">UNII</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="precision.fda.gov"><a rel="nofollow" class="external text" href="https://precision.fda.gov/uniisearch/srs/unii/EGX657432I">EGX657432I</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/KEGG" title="KEGG">KEGG</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.kegg.jp"><a rel="nofollow" class="external text" href="https://www.kegg.jp/entry/D08505">D08505</a></span></li><li>as salt: <span title="www.kegg.jp"><a rel="nofollow" class="external text" href="https://www.kegg.jp/entry/D01798">D01798</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ChEBI" title="ChEBI">ChEBI</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.ebi.ac.uk"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/chebi/searchId.do?chebiId=CHEBI:59560">CHEBI:59560</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li><li>as salt: <span title="www.ebi.ac.uk"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/chebi/searchId.do?chebiId=CHEBI:32120">CHEBI:32120</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ChEMBL" title="ChEMBL">ChEMBL</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.ebi.ac.uk"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/chembl/explore/compound/ChEMBL1201774">ChEMBL1201774</a></span><sup> <span typeof="mw:File"><span><img alt="☒" src="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/7px-X_mark.svg.png" decoding="async" width="7" height="8" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/11px-X_mark.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/14px-X_mark.svg.png 2x" data-file-width="525" data-file-height="600" /></span></span><span style="display:none">N</span></sup></li><li>as salt: <span title="www.ebi.ac.uk"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/chembl/explore/compound/ChEMBL1201775">ChEMBL1201775</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Protein_Data_Bank" title="Protein Data Bank">PDB ligand</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li>H4B (<span title="www.ebi.ac.uk"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/pdbe-srv/PDBeXplore/ligand/?ligand=H4B">PDBe</a></span>, <span title="www.rcsb.org"><a rel="nofollow" class="external text" href="https://www.rcsb.org/ligand/H4B">RCSB PDB</a></span>)</li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/CompTox_Chemicals_Dashboard" title="CompTox Chemicals Dashboard">CompTox Dashboard</a> <span style="font-weight:normal">(<abbr title="U.S. Environmental Protection Agency">EPA</abbr>)</span></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="comptox.epa.gov"><a rel="nofollow" class="external text" href="https://comptox.epa.gov/dashboard/chemical/details/DTXSID1041138">DTXSID1041138</a> <span class="mw-valign-text-top noprint" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q419808#P3117" title="Edit this at Wikidata"><img alt="Edit this at Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ECHA_InfoCard" class="mw-redirect" title="ECHA InfoCard"><span title="echa.europa.eu">ECHA InfoCard</span></a></th><td class="infobox-data"><a rel="nofollow" class="external text" href="https://echa.europa.eu/substance-information/-/substanceinfo/100.164.121">100.164.121</a> <span class="mw-valign-text-top noprint" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q419808#P2566" title="Edit this at Wikidata"><img alt="Edit this at Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Chemical and physical data</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Chemical_formula" title="Chemical formula">Formula</a></th><td class="infobox-data"><span title="Carbon">C</span><sub>9</sub><span title="Hydrogen">H</span><sub>15</sub><span title="Nitrogen">N</span><sub>5</sub><span title="Oxygen">O</span><sub>3</sub></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Molar_mass" title="Molar mass">Molar mass</a></th><td class="infobox-data"><span class="nowrap"><span data-sort-value="7002241251000000000♠"></span>241.251</span> g·mol<sup>−1</sup></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;">3D model (<a href="/wiki/JSmol" class="mw-redirect" title="JSmol">JSmol</a>)</th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="chemapps.stolaf.edu (3D interactive model)"><a rel="nofollow" class="external text" href="https://chemapps.stolaf.edu/jmol/jmol.php?model=CC%28C%28C1CNC2%3DC%28N1%29C%28%3DO%29N%3DC%28N2%29N%29O%29O">Interactive image</a></span></li></ul></div></td></tr><tr><td colspan="2" class="infobox-full-data"><div class="collapsible-list mw-collapsible mw-collapsed" style="text-align: left;"> <div style="line-height: 1.6em; font-weight: bold;"><div><a href="/wiki/Simplified_molecular-input_line-entry_system" class="mw-redirect" title="Simplified molecular-input line-entry system">SMILES</a></div></div> <ul class="mw-collapsible-content" style="margin-top: 0; margin-bottom: 0; line-height: inherit; list-style: none; margin-left: 0; word-break:break-all;"><li style="line-height: inherit; margin: 0"><div style="word-wrap:break-word; text-indent:-1.5em; text-align:left; padding-left:1.5em; font-size:97%; line-height:120%;">CC(C(C1CNC2=C(N1)C(=O)N=C(N2)N)O)O</div></li></ul> </div></td></tr><tr><td colspan="2" class="infobox-full-data"><div class="collapsible-list mw-collapsible mw-collapsed" style="text-align: left;"> <div style="line-height: 1.6em; font-weight: bold;"><div><a href="/wiki/International_Chemical_Identifier" title="International Chemical Identifier">InChI</a></div></div> <ul class="mw-collapsible-content" style="margin-top: 0; margin-bottom: 0; line-height: inherit; list-style: none; margin-left: 0; word-break:break-all;"><li style="line-height: inherit; margin: 0"><div style="word-wrap:break-word; text-indent:-1.5em; text-align:left; padding-left:1.5em; font-size:97%; line-height:120%;">InChI=1S/C9H15N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3-4,6,12,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,4+,6-/m0/s1<sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></div></li><li style="line-height: inherit; margin: 0"><div style="word-wrap:break-word; text-indent:-1.5em; text-align:left; padding-left:1.5em; font-size:97%; line-height:120%;">Key:FNKQXYHWGSIFBK-RPDRRWSUSA-N<sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></div></li></ul> </div></td></tr><tr><td colspan="2" class="infobox-below"><style data-mw-deduplicate="TemplateStyles:r886047488">.mw-parser-output .nobold{font-weight:normal}</style><span class="nobold"> <sup><span typeof="mw:File"><span><img alt="☒" src="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/7px-X_mark.svg.png" decoding="async" width="7" height="8" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/11px-X_mark.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/14px-X_mark.svg.png 2x" data-file-width="525" data-file-height="600" /></span></span><span style="display:none">N</span><span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup> <a href="/wiki/Wikipedia:WikiProject_Chemicals/Chembox_validation" title="Wikipedia:WikiProject Chemicals/Chembox validation">(what is this?)</a></span><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r886047488" /><span class="nobold">  <span class="reflink plainlinks nourlexpansion"><a class="external text" href="https://en.wikipedia.org/w/index.php?title=Special:ComparePages&rev1=470604083&page2=Tetrahydrobiopterin">(verify)</a></span></span></td></tr></tbody></table> <p><b>Tetrahydrobiopterin</b> (<b>BH<sub>4</sub></b>, <b>THB</b>), also known as <b>sapropterin</b> (<a href="/wiki/International_nonproprietary_name" title="International nonproprietary name">INN</a>),<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> is a <a href="/wiki/Cofactor_(biochemistry)" title="Cofactor (biochemistry)">cofactor</a> of the three <a href="/wiki/Aromatic_amino_acid_hydroxylase" class="mw-redirect" title="Aromatic amino acid hydroxylase">aromatic amino acid hydroxylase</a> enzymes,<sup id="cite_ref-Cara_7-0" class="reference"><a href="#cite_note-Cara-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> used in the degradation of amino acid <a href="/wiki/Phenylalanine" title="Phenylalanine">phenylalanine</a> and in the <a href="/wiki/Biosynthesis" title="Biosynthesis">biosynthesis</a> of the <a href="/wiki/Neurotransmitter" title="Neurotransmitter">neurotransmitters</a> <a href="/wiki/Serotonin" title="Serotonin">serotonin</a> (5-hydroxytryptamine, 5-HT), <a href="/wiki/Melatonin" title="Melatonin">melatonin</a>, <a href="/wiki/Dopamine" title="Dopamine">dopamine</a>, <a href="/wiki/Norepinephrine" title="Norepinephrine">norepinephrine</a> (noradrenaline), <a href="/wiki/Epinephrine" class="mw-redirect" title="Epinephrine">epinephrine</a> (adrenaline), and is a cofactor for the production of <a href="/wiki/Nitric_oxide" title="Nitric oxide">nitric oxide</a> (NO) by the nitric oxide synthases.<sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> Chemically, its structure is that of a (dihydropteridine reductase) reduced <a href="/wiki/Pteridine" title="Pteridine">pteridine</a> derivative (quinonoid dihydrobiopterin).<sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (March 2020)">citation needed</span></a></i>]</sup> </p><p>Tetrahydrobiopterin is available as a tablet for <a href="/wiki/Oral_administration" title="Oral administration">oral administration</a> in the form of sapropterin dihydrochloride (BH4*2HCL).<sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kuvan_FDA_label_3-1" class="reference"><a href="#cite_note-Kuvan_FDA_label-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kuvan_EPAR_4-1" class="reference"><a href="#cite_note-Kuvan_EPAR-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> It was approved for use in the United States as a tablet in December 2007<sup id="cite_ref-12" class="reference"><a href="#cite_note-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-BioMarin_Kuvan_13-0" class="reference"><a href="#cite_note-BioMarin_Kuvan-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> and as a powder in December 2013.<sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-BioMarin_Kuvan_13-1" class="reference"><a href="#cite_note-BioMarin_Kuvan-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> It was approved for use in the European Union in December 2008,<sup id="cite_ref-Kuvan_EPAR_4-2" class="reference"><a href="#cite_note-Kuvan_EPAR-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Canada in April 2010,<sup id="cite_ref-Kuvan_CA_Product_information_2-1" class="reference"><a href="#cite_note-Kuvan_CA_Product_information-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> and Japan in July 2008.<sup id="cite_ref-BioMarin_Kuvan_13-2" class="reference"><a href="#cite_note-BioMarin_Kuvan-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> It is sold under the brand names <b>Kuvan</b> and <b>Biopten</b>.<sup id="cite_ref-Kuvan_EPAR_4-3" class="reference"><a href="#cite_note-Kuvan_EPAR-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kuvan_FDA_label_3-2" class="reference"><a href="#cite_note-Kuvan_FDA_label-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-BioMarin_Kuvan_13-3" class="reference"><a href="#cite_note-BioMarin_Kuvan-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> The typical cost of treating a patient with Kuvan is <span style="white-space: nowrap">US$100,000</span> per year.<sup id="cite_ref-15" class="reference"><a href="#cite_note-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> <a href="/wiki/BioMarin" class="mw-redirect" title="BioMarin">BioMarin</a> holds the patent for Kuvan until at least 2024, but <a href="/wiki/Par_Pharmaceutical" class="mw-redirect" title="Par Pharmaceutical">Par Pharmaceutical</a> has a right to produce a generic version by 2020.<sup id="cite_ref-16" class="reference"><a href="#cite_note-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Medical_uses">Medical uses</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=1" title="Edit section: Medical uses"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Sapropterin is indicated in <a href="/wiki/Tetrahydrobiopterin_deficiency" title="Tetrahydrobiopterin deficiency">tetrahydrobiopterin deficiency</a> caused by <a href="/wiki/GTP_cyclohydrolase_I" title="GTP cyclohydrolase I">GTP cyclohydrolase I</a> (GTPCH) deficiency, or <a href="/wiki/6-pyruvoyltetrahydropterin_synthase#Clinical_significance" class="mw-redirect" title="6-pyruvoyltetrahydropterin synthase">6-pyruvoyltetrahydropterin synthase</a> (PTPS) deficiency.<sup id="cite_ref-17" class="reference"><a href="#cite_note-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> Also, BH4*2HCL is FDA approved for use in <a href="/wiki/Phenylketonuria" title="Phenylketonuria">phenylketonuria</a> (PKU), along with dietary measures.<sup id="cite_ref-NIH2013Tx_18-0" class="reference"><a href="#cite_note-NIH2013Tx-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> However, most people with PKU have little or no benefit from BH4*2HCL.<sup id="cite_ref-19" class="reference"><a href="#cite_note-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Adverse_effects">Adverse effects</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=2" title="Edit section: Adverse effects"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The most common <a href="/wiki/Adverse_effects" class="mw-redirect" title="Adverse effects">adverse effects</a>, observed in more than 10% of people, include headache and a running or obstructed nose. Diarrhea and vomiting are also relatively common, seen in at least 1% of people.<sup id="cite_ref-AustriaCodex_20-0" class="reference"><a href="#cite_note-AustriaCodex-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Interactions">Interactions</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=3" title="Edit section: Interactions"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>No interaction studies have been conducted. Because of its mechanism, tetrahydrobiopterin might interact with <a href="/wiki/Dihydrofolate_reductase_inhibitor" title="Dihydrofolate reductase inhibitor">dihydrofolate reductase inhibitors</a> like <a href="/wiki/Methotrexate" title="Methotrexate">methotrexate</a> and <a href="/wiki/Trimethoprim" title="Trimethoprim">trimethoprim</a>, and NO-enhancing drugs like <a href="/wiki/Nitroglycerin" title="Nitroglycerin">nitroglycerin</a>, <a href="/wiki/Molsidomine" title="Molsidomine">molsidomine</a>, <a href="/wiki/Minoxidil" title="Minoxidil">minoxidil</a>, and <a href="/wiki/PDE5_inhibitors" class="mw-redirect" title="PDE5 inhibitors">PDE5 inhibitors</a>. Combination of tetrahydrobiopterin with <a href="/wiki/Levodopa" title="Levodopa">levodopa</a> can lead to increased excitability.<sup id="cite_ref-AustriaCodex_20-1" class="reference"><a href="#cite_note-AustriaCodex-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Functions">Functions</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=4" title="Edit section: Functions"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Tetrahydrobiopterin has multiple roles in human biochemistry. The major one is to convert amino acids such as phenylalanine, tyrosine, and tryptophan to precursors of dopamine and serotonin, major <a href="/wiki/Monoamine" class="mw-redirect" title="Monoamine">monoamine</a> neurotransmitters.<sup id="cite_ref-21" class="reference"><a href="#cite_note-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> It works as a <a href="/wiki/Cofactor_(biochemistry)" title="Cofactor (biochemistry)">cofactor</a>, being required for an enzyme's activity as a catalyst, mainly <a href="/wiki/Hydroxylase" class="mw-redirect" title="Hydroxylase">hydroxylases</a>.<sup id="cite_ref-Cara_7-1" class="reference"><a href="#cite_note-Cara-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Cofactor_for_tryptophan_hydroxylases">Cofactor for tryptophan hydroxylases</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=5" title="Edit section: Cofactor for tryptophan hydroxylases"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236090951" /><div role="note" class="hatnote navigation-not-searchable">Further information: <a href="/wiki/Tryptophan_hydroxylase" title="Tryptophan hydroxylase">Tryptophan hydroxylase</a></div> <p>Tetrahydrobiopterin is a cofactor for <a href="/wiki/Tryptophan_hydroxylase" title="Tryptophan hydroxylase">tryptophan hydroxylase</a> (TPH) for the conversion of <a href="/wiki/L-tryptophan" class="mw-redirect" title="L-tryptophan">L-tryptophan</a> (TRP) to <a href="/wiki/5-hydroxytryptophan" class="mw-redirect" title="5-hydroxytryptophan">5-hydroxytryptophan</a> (5-HTP). </p> <div class="mw-heading mw-heading3"><h3 id="Cofactor_for_phenylalanine_hydroxylase">Cofactor for phenylalanine hydroxylase</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=6" title="Edit section: Cofactor for phenylalanine hydroxylase"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Phenylalanine_hydroxylase" title="Phenylalanine hydroxylase">Phenylalanine hydroxylase</a> (PAH) catalyses the conversion of <a href="/wiki/L-phenylalanine" class="mw-redirect" title="L-phenylalanine">L-phenylalanine</a> (PHE) to <a href="/wiki/L-tyrosine" class="mw-redirect" title="L-tyrosine">L-tyrosine</a> (TYR). Therefore, a deficiency in tetrahydrobiopterin can cause a toxic buildup of L-phenylalanine, which manifests as the severe neurological issues seen in <a href="/wiki/Phenylketonuria" title="Phenylketonuria">phenylketonuria</a>. </p> <div class="mw-heading mw-heading3"><h3 id="Cofactor_for_tyrosine_hydroxylase">Cofactor for tyrosine hydroxylase</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=7" title="Edit section: Cofactor for tyrosine hydroxylase"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Tyrosine_hydroxylase" title="Tyrosine hydroxylase">Tyrosine hydroxylase</a> (TH) catalyses the conversion of L-tyrosine to <a href="/wiki/L-DOPA" title="L-DOPA">L-DOPA</a> (DOPA), which is the precursor for <a href="/wiki/Dopamine" title="Dopamine">dopamine</a>. Dopamine is a vital <a href="/wiki/Neurotransmitter" title="Neurotransmitter">neurotransmitter</a>, and is the precursor of <a href="/wiki/Norepinephrine" title="Norepinephrine">norepinephrine</a> and <a href="/wiki/Epinephrine" class="mw-redirect" title="Epinephrine">epinephrine</a>. Thus, a deficiency of BH4 can lead to systemic deficiencies of dopamine, norepinephrine, and epinephrine. In fact, one of the primary conditions that can result from GTPCH-related BH4 deficiency is <a href="/wiki/Dopamine-responsive_dystonia" title="Dopamine-responsive dystonia">dopamine-responsive dystonia</a>;<sup id="cite_ref-22" class="reference"><a href="#cite_note-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> currently, this condition is typically treated with <a href="/wiki/Carbidopa/levodopa" title="Carbidopa/levodopa">carbidopa/levodopa</a>, which directly restores dopamine levels within the brain. </p> <div class="mw-heading mw-heading3"><h3 id="Cofactor_for_nitric_oxide_synthase">Cofactor for nitric oxide synthase</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=8" title="Edit section: Cofactor for nitric oxide synthase"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Nitric_oxide_synthase" title="Nitric oxide synthase">Nitric oxide synthase</a> (NOS) catalyses the conversion of a guanidino nitrogen of <a href="/wiki/L-arginine" class="mw-redirect" title="L-arginine">L-arginine</a> (L-Arg) to <a href="/wiki/Nitric_oxide" title="Nitric oxide">nitric oxide</a> (NO). Among other things, nitric oxide is involved in <a href="/wiki/Vasodilation" title="Vasodilation">vasodilation</a>, which improves systematic blood flow. The role of BH4 in this enzymatic process is so critical that some research points to a deficiency of BH4 – and thus, of nitric oxide – as being a core cause of the neurovascular dysfunction that is the hallmark of circulation-related diseases such as <a href="/wiki/Diabetes" title="Diabetes">diabetes</a>.<sup id="cite_ref-23" class="reference"><a href="#cite_note-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup> As a co-factor for nitric oxide synthase, tetrahydrobiopterin supplementation has shown beneficial results for the treatment of <a href="/wiki/Endothelial_dysfunction" title="Endothelial dysfunction">endothelial dysfunction</a> in animal experiments and clinical trials, although the tendency of BH4 to become oxidized to BH2 remains a problem.<sup id="cite_ref-pmid29596860_24-0" class="reference"><a href="#cite_note-pmid29596860-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Cofactor_for_ether_lipid_oxidase">Cofactor for ether lipid oxidase</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=9" title="Edit section: Cofactor for ether lipid oxidase"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Ether lipid oxidase (<a href="/wiki/Alkylglycerol_monooxygenase" title="Alkylglycerol monooxygenase">alkylglycerol monooxygenase</a>, AGMO) catalyses the conversion of <a href="/w/index.php?title=1-alkyl-sn-glycerol&action=edit&redlink=1" class="new" title="1-alkyl-sn-glycerol (page does not exist)">1-alkyl-sn-glycerol</a> to <a href="/w/index.php?title=1-hydroxyalkyl-sn-glycerol&action=edit&redlink=1" class="new" title="1-hydroxyalkyl-sn-glycerol (page does not exist)">1-hydroxyalkyl-sn-glycerol</a>. </p> <div class="mw-heading mw-heading2"><h2 id="History">History</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=10" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Tetrahydrobiopterin was discovered to play a role as an enzymatic cofactor. The first enzyme found to use tetrahydrobiopterin is <a href="/wiki/Phenylalanine_hydroxylase" title="Phenylalanine hydroxylase">phenylalanine hydroxylase</a> (PAH).<sup id="cite_ref-25" class="reference"><a href="#cite_note-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Biosynthesis_and_recycling">Biosynthesis and recycling</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=11" title="Edit section: Biosynthesis and recycling"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Tetrahydrobiopterin is biosynthesized from <a href="/wiki/Guanosine_triphosphate" title="Guanosine triphosphate">guanosine triphosphate</a> (GTP) by three chemical reactions mediated by the enzymes <a href="/wiki/GTP_cyclohydrolase_I" title="GTP cyclohydrolase I">GTP cyclohydrolase I</a> (GTPCH), <a href="/wiki/6-pyruvoyltetrahydropterin_synthase" class="mw-redirect" title="6-pyruvoyltetrahydropterin synthase">6-pyruvoyltetrahydropterin synthase</a> (PTPS), and <a href="/wiki/Sepiapterin_reductase" title="Sepiapterin reductase">sepiapterin reductase</a> (SR).<sup id="cite_ref-pmid10727395_26-0" class="reference"><a href="#cite_note-pmid10727395-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup> </p><p>BH4 can be oxidized by one or two electron reactions, to generate BH4 or BH3 radical and BH2, respectively. Research shows that ascorbic acid (also known as ascorbate or <a href="/wiki/Vitamin_C" title="Vitamin C">vitamin C</a>) can reduce BH3 radical into BH4,<sup id="cite_ref-27" class="reference"><a href="#cite_note-27"><span class="cite-bracket">[</span>27<span class="cite-bracket">]</span></a></sup> preventing the BH3 radical from reacting with other free radicals (<a href="/wiki/Superoxide" title="Superoxide">superoxide</a> and <a href="/wiki/Peroxynitrite" title="Peroxynitrite">peroxynitrite</a> specifically). Without this recycling process, uncoupling of the <a href="/wiki/Endothelial_nitric_oxide_synthase" class="mw-redirect" title="Endothelial nitric oxide synthase">endothelial nitric oxide synthase</a> (eNOS) enzyme and reduced bioavailability of the <a href="/wiki/Vasodilator" class="mw-redirect" title="Vasodilator">vasodilator</a> <a href="/wiki/Nitric_oxide" title="Nitric oxide">nitric oxide</a> occur, creating a form of <a href="/wiki/Endothelial_dysfunction" title="Endothelial dysfunction">endothelial dysfunction</a>.<sup id="cite_ref-28" class="reference"><a href="#cite_note-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup> Ascorbic acid is oxidized to <a href="/wiki/Dehydroascorbic_acid" title="Dehydroascorbic acid">dehydroascorbic acid</a> during this process, although it can be recycled back to ascorbic acid. </p><p>Folic acid and its metabolites seem to be particularly important in the recycling of BH4 and NOS coupling.<sup id="cite_ref-29" class="reference"><a href="#cite_note-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Research">Research</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=12" title="Edit section: Research"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Other than PKU studies, tetrahydrobiopterin has participated in clinical trials studying other approaches to solving conditions resultant from a deficiency of tetrahydrobiopterin. These include <a href="/wiki/Autism" title="Autism">autism</a>, <a href="/wiki/Depression_(mood)" title="Depression (mood)">depression</a>,<sup id="cite_ref-30" class="reference"><a href="#cite_note-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup> <a href="/wiki/ADHD" class="mw-redirect" title="ADHD">ADHD</a>, <a href="/wiki/Hypertension" title="Hypertension">hypertension</a>, endothelial dysfunction, and <a href="/wiki/Chronic_kidney_disease" title="Chronic kidney disease">chronic kidney disease</a>.<sup id="cite_ref-31" class="reference"><a href="#cite_note-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-32" class="reference"><a href="#cite_note-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup> Experimental studies suggest that tetrahydrobiopterin regulates deficient production of nitric oxide in cardiovascular disease states, and contributes to the response to inflammation and injury, for example in pain due to nerve injury. A 2015 BioMarin-funded study of PKU patients found that those who responded to tetrahydrobiopterin also showed a reduction of ADHD symptoms.<sup id="cite_ref-33" class="reference"><a href="#cite_note-33"><span class="cite-bracket">[</span>33<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Depression">Depression</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=13" title="Edit section: Depression"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In psychiatry, tetrahydrobiopterin has been hypothesized to be involved in the pathophysiology of depression, although evidence is inconclusive to date.<sup id="cite_ref-34" class="reference"><a href="#cite_note-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Autism">Autism</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=14" title="Edit section: Autism"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In 1997, a small pilot study was published on the efficacy of tetrahydrobiopterin (BH4) on relieving the symptoms of autism, which concluded that it "might be useful for a subgroup of children with autism" and that double-blind trials are needed, as are trials which measure outcomes over a longer period of time.<sup id="cite_ref-35" class="reference"><a href="#cite_note-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup> In 2010, <a href="/wiki/Richard_E._Frye" title="Richard E. Frye">Frye</a> et al. published a paper which concluded that it was safe, and also noted that "several clinical trials have suggested that treatment with BH4 improves ASD symptomatology in some individuals."<sup id="cite_ref-36" class="reference"><a href="#cite_note-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Cardiovascular_disease">Cardiovascular disease</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=15" title="Edit section: Cardiovascular disease"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Since <a href="/wiki/Nitric_oxide" title="Nitric oxide">nitric oxide</a> production is important in regulation of blood pressure and blood flow, thereby playing a significant role in cardiovascular diseases, tetrahydrobiopterin is a potential therapeutic target. In the endothelial cell lining of blood vessels, <a href="/wiki/Endothelial_nitric_oxide_synthase" class="mw-redirect" title="Endothelial nitric oxide synthase">endothelial nitric oxide synthase</a> is dependent on tetrahydrobiopterin availability.<sup id="cite_ref-37" class="reference"><a href="#cite_note-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> Increasing tetrahydrobiopterin in endothelial cells by augmenting the levels of the biosynthetic enzyme GTPCH can maintain endothelial nitric oxide synthase function in experimental models of disease states such as diabetes,<sup id="cite_ref-38" class="reference"><a href="#cite_note-38"><span class="cite-bracket">[</span>38<span class="cite-bracket">]</span></a></sup> atherosclerosis, and hypoxic pulmonary hypertension.<sup id="cite_ref-39" class="reference"><a href="#cite_note-39"><span class="cite-bracket">[</span>39<span class="cite-bracket">]</span></a></sup> However, treatment of people with existing coronary artery disease with oral tetrahydrobiopterin is limited by oxidation of tetrahydrobiopterin to the inactive form, <a href="/wiki/Dihydrobiopterin" title="Dihydrobiopterin">dihydrobiopterin</a>, with little benefit on vascular function.<sup id="cite_ref-40" class="reference"><a href="#cite_note-40"><span class="cite-bracket">[</span>40<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Neuroprotection_in_prenatal_hypoxia">Neuroprotection in prenatal hypoxia</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=16" title="Edit section: Neuroprotection in prenatal hypoxia"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Depletion of tetrahydrobiopterin occurs in the hypoxic brain and leads to toxin production. Preclinical studies in mice reveal that treatment with oral tetrahydrobiopterin therapy mitigates the toxic effects of hypoxia on the developing brain, specifically improving white matter development in hypoxic animals.<sup id="cite_ref-41" class="reference"><a href="#cite_note-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Programmed_cell_death">Programmed cell death</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=17" title="Edit section: Programmed cell death"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>GTPCH (GCH1) and tetrahydrobiopterin were found to have a secondary role protecting against <a href="/wiki/Cell_death" title="Cell death">cell death</a> by <a href="/wiki/Ferroptosis" title="Ferroptosis">ferroptosis</a> in cellular models by limiting the formation of toxic <a href="/wiki/Lipid" title="Lipid">lipid</a> peroxides.<sup id="cite_ref-42" class="reference"><a href="#cite_note-42"><span class="cite-bracket">[</span>42<span class="cite-bracket">]</span></a></sup> Tetrahydrobiopterin acts as a potent, diffusable <a href="/wiki/Antioxidant" title="Antioxidant">antioxidant</a> that resists <a href="/wiki/Oxidative_stress" title="Oxidative stress">oxidative stress</a><sup id="cite_ref-43" class="reference"><a href="#cite_note-43"><span class="cite-bracket">[</span>43<span class="cite-bracket">]</span></a></sup> and enables <a href="/wiki/Cancer" title="Cancer">cancer</a> cell survival via promotion of angiogenesis.<sup id="cite_ref-44" class="reference"><a href="#cite_note-44"><span class="cite-bracket">[</span>44<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=18" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-Drugs.com_pregnancy-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-Drugs.com_pregnancy_1-0">^</a></b></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://www.drugs.com/pregnancy/sapropterin.html">"Sapropterin (Kuvan) Use During Pregnancy"</a>. <i>Drugs.com</i>. 17 May 2019<span class="reference-accessdate">. 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(October 2017). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5639803">"The complete European guidelines on phenylketonuria: diagnosis and treatment"</a>. <i>Orphanet Journal of Rare Diseases</i>. <b>12</b> (1): 162. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1186%2Fs13023-017-0685-2">10.1186/s13023-017-0685-2</a></span>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5639803">5639803</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/29025426">29025426</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Orphanet+Journal+of+Rare+Diseases&rft.atitle=The+complete+European+guidelines+on+phenylketonuria%3A+diagnosis+and+treatment&rft.volume=12&rft.issue=1&rft.pages=162&rft.date=2017-10&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC5639803%23id-name%3DPMC&rft_id=info%3Apmid%2F29025426&rft_id=info%3Adoi%2F10.1186%2Fs13023-017-0685-2&rft.aulast=van+Wegberg&rft.aufirst=AM&rft.au=MacDonald%2C+A&rft.au=Ahring%2C+K&rft.au=B%C3%A9langer-Quintana%2C+A&rft.au=Blau%2C+N&rft.au=Bosch%2C+AM&rft.au=Burlina%2C+A&rft.au=Campistol%2C+J&rft.au=Feillet%2C+F&rft.au=Gi%C5%BCewska%2C+M&rft.au=Huijbregts%2C+SC&rft.au=Kearney%2C+S&rft.au=Leuzzi%2C+V&rft.au=Maillot%2C+F&rft.au=Muntau%2C+AC&rft.au=van+Rijn%2C+M&rft.au=Trefz%2C+F&rft.au=Walter%2C+JH&rft.au=van+Spronsen%2C+FJ&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC5639803&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetrahydrobiopterin" class="Z3988"></span></li></ul> </div> <div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetrahydrobiopterin&action=edit&section=20" title="Edit section: External links"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://druginfo.nlm.nih.gov/drugportal/name/sapropterin">"Sapropterin"</a>. <i>Drug Information Portal</i>. U.S. National Library of Medicine.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Drug+Information+Portal&rft.atitle=Sapropterin&rft_id=https%3A%2F%2Fdruginfo.nlm.nih.gov%2Fdrugportal%2Fname%2Fsapropterin&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetrahydrobiopterin" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://druginfo.nlm.nih.gov/drugportal/name/sapropterin%20dihydrochloride">"Sapropterin dihydrochloride"</a>. <i>Drug Information Portal</i>. U.S. National Library of Medicine.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Drug+Information+Portal&rft.atitle=Sapropterin+dihydrochloride&rft_id=https%3A%2F%2Fdruginfo.nlm.nih.gov%2Fdrugportal%2Fname%2Fsapropterin%2520dihydrochloride&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetrahydrobiopterin" class="Z3988"></span></li></ul> <div class="navbox-styles"><style data-mw-deduplicate="TemplateStyles:r1129693374">.mw-parser-output .hlist dl,.mw-parser-output .hlist ol,.mw-parser-output .hlist ul{margin:0;padding:0}.mw-parser-output .hlist dd,.mw-parser-output .hlist dt,.mw-parser-output .hlist li{margin:0;display:inline}.mw-parser-output .hlist.inline,.mw-parser-output .hlist.inline dl,.mw-parser-output .hlist.inline ol,.mw-parser-output .hlist.inline ul,.mw-parser-output .hlist dl dl,.mw-parser-output .hlist dl ol,.mw-parser-output .hlist dl ul,.mw-parser-output .hlist ol dl,.mw-parser-output .hlist ol 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.navbox{display:none!important}}</style></div><div role="navigation" class="navbox" aria-labelledby="Enzyme_cofactors70" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374" /><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Enzyme_cofactors" title="Template:Enzyme cofactors"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Enzyme_cofactors" title="Template talk:Enzyme cofactors"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Enzyme_cofactors" title="Special:EditPage/Template:Enzyme cofactors"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Enzyme_cofactors70" style="font-size:114%;margin:0 4em"><a href="/wiki/Enzyme" title="Enzyme">Enzyme</a> <a href="/wiki/Cofactor_(biochemistry)" title="Cofactor (biochemistry)">cofactors</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%">Active forms</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Vitamin" title="Vitamin">vitamins</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Thiamine_pyrophosphate" title="Thiamine pyrophosphate">TPP / ThDP</a> (B<sub>1</sub>)</li> <li><a href="/wiki/Flavin_mononucleotide" title="Flavin mononucleotide">FMN</a>, <a href="/wiki/Flavin_adenine_dinucleotide" title="Flavin adenine dinucleotide">FAD</a> (B<sub>2</sub>)</li> <li><a href="/wiki/Nicotinamide_adenine_dinucleotide" title="Nicotinamide adenine dinucleotide">NAD<sup>+</sup></a>, <a href="/wiki/Nicotinamide_adenine_dinucleotide" title="Nicotinamide adenine dinucleotide">NADH</a>, <a href="/wiki/Nicotinamide_adenine_dinucleotide_phosphate" title="Nicotinamide adenine dinucleotide phosphate">NADP<sup>+</sup></a>, <a href="/wiki/Nicotinamide_adenine_dinucleotide_phosphate" title="Nicotinamide adenine dinucleotide phosphate">NADPH</a> (B<sub>3</sub>)</li> <li><a href="/wiki/Coenzyme_A" title="Coenzyme A">Coenzyme A</a> (B<sub>5</sub>)</li> <li><a href="/wiki/Pyridoxal_phosphate" title="Pyridoxal phosphate">PLP / P5P</a> (B<sub>6</sub>)</li> <li><a href="/wiki/Biotin" title="Biotin">Biotin</a> (B<sub>7</sub>)</li> <li><a href="/wiki/Tetrahydrofolic_acid" title="Tetrahydrofolic acid">THFA / H<sub>4</sub>FA</a>, <a href="/wiki/Dihydrofolic_acid" title="Dihydrofolic acid">DHFA / H<sub>2</sub>FA</a>, <a href="/wiki/5,10-Methylenetetrahydrofolate" title="5,10-Methylenetetrahydrofolate">MTHF</a> (B<sub>9</sub>)</li> <li><a href="/wiki/Adenosylcobalamin" title="Adenosylcobalamin">AdoCbl</a>, <a href="/wiki/Methylcobalamin" title="Methylcobalamin">MeCbl</a> (B<sub>12</sub>)</li> <li><a href="/wiki/Vitamin_C" title="Vitamin C">Ascorbic acid</a> (C)</li> <li><a href="/wiki/Phytomenadione" title="Phytomenadione">Phylloquinone</a> (K<sub>1</sub>), <a href="/wiki/Vitamin_K" title="Vitamin K">Menaquinone</a> (K<sub>2</sub>)</li> <li><a href="/wiki/Coenzyme_F420" title="Coenzyme F420">Coenzyme F420</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Cofactor_(biochemistry)#Non-vitamins" title="Cofactor (biochemistry)">non-vitamins</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Adenosine_triphosphate" title="Adenosine triphosphate">ATP</a></li> <li><a href="/wiki/Cytidine_triphosphate" title="Cytidine triphosphate">CTP</a></li> <li><a href="/wiki/S-Adenosyl_methionine" title="S-Adenosyl methionine">SAMe</a></li> <li><a href="/wiki/3%27-Phosphoadenosine-5%27-phosphosulfate" title="3'-Phosphoadenosine-5'-phosphosulfate">PAPS</a></li> <li><a href="/wiki/Glutathione" title="Glutathione">GSH</a></li> <li><a href="/wiki/Coenzyme_B" title="Coenzyme B">Coenzyme B</a></li> <li><a href="/wiki/Cofactor_F430" title="Cofactor F430">Cofactor F430</a></li> <li><a href="/wiki/Coenzyme_M" title="Coenzyme M">Coenzyme M</a></li> <li><a href="/wiki/Coenzyme_Q10" title="Coenzyme Q10">Coenzyme Q</a></li> <li><a href="/wiki/Heme" title="Heme">Heme / Haem</a> (<a href="/wiki/Heme_A" title="Heme A">A</a>, <a href="/wiki/Heme_B" title="Heme B">B</a>, <a href="/wiki/Heme_C" title="Heme C">C</a>, <a href="/wiki/Heme_O" title="Heme O">O</a>)</li> <li><a href="/wiki/Lipoic_acid" title="Lipoic acid">Lipoic Acid</a></li> <li><a href="/wiki/Methanofuran" title="Methanofuran">Methanofuran</a></li> <li><a href="/wiki/Molybdopterin" title="Molybdopterin">Molybdopterin</a></li> <li><a href="/wiki/Mycofactocin" title="Mycofactocin">Mycofactocin</a></li> <li><a href="/wiki/Pyrroloquinoline_quinone" title="Pyrroloquinoline quinone">PQQ</a></li> <li><a class="mw-selflink selflink">THB / BH<sub>4</sub></a></li> <li><a href="/wiki/Tetrahydromethanopterin" title="Tetrahydromethanopterin">THMPT / H<sub>4</sub>MPT</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mineral_(nutrient)" title="Mineral (nutrient)">metal ions</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Calcium" title="Calcium">Ca<sup>2+</sup></a></li> <li><a href="/wiki/Copper" title="Copper">Cu<sup>2+</sup></a></li> <li><a href="/wiki/Iron" title="Iron">Fe<sup>2+</sup>, Fe<sup>3+</sup></a></li> <li><a href="/wiki/Magnesium" title="Magnesium">Mg<sup>2+</sup></a></li> <li><a href="/wiki/Manganese" title="Manganese">Mn<sup>2+</sup></a></li> <li><a href="/wiki/Molybdenum" title="Molybdenum">Mo</a></li> <li><a href="/wiki/Nickel" title="Nickel">Ni<sup>2+</sup></a></li> <li><a href="/wiki/Zinc" title="Zinc">Zn<sup>2+</sup></a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Base forms</th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/Vitamin" title="Vitamin">vitamins</a></i>: <i>see <a href="/wiki/Template:Vitamins" title="Template:Vitamins">vitamins</a></i></li></ul> </div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374" /><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235" /></div><div role="navigation" class="navbox" aria-labelledby="Other_alimentary_tract_and_metabolism_products_(A16)124" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374" /><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1239400231" /><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Other_alimentary_tract_and_metabolism_products" title="Template:Other alimentary tract and metabolism products"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Other_alimentary_tract_and_metabolism_products" title="Template talk:Other alimentary tract and metabolism products"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Other_alimentary_tract_and_metabolism_products" title="Special:EditPage/Template:Other alimentary tract and metabolism products"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Other_alimentary_tract_and_metabolism_products_(A16)124" style="font-size:114%;margin:0 4em">Other <a href="/wiki/Alimentary_tract" class="mw-redirect" title="Alimentary tract">alimentary tract</a> and <a href="/wiki/Metabolism" title="Metabolism">metabolism</a> products (<a href="/wiki/ATC_code_A16" title="ATC code A16">A16</a>)</div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Amino_acid" title="Amino acid">Amino acids</a> and derivatives</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/S-Adenosyl_methionine" title="S-Adenosyl methionine">Ademetionine</a></li> <li><a href="/wiki/Betaine" title="Betaine">Betaine</a></li> <li><a href="/wiki/Carglumic_acid" title="Carglumic acid">Carglumic acid</a></li> <li><a href="/wiki/Glutamine" title="Glutamine">Glutamine</a></li> <li><a href="/wiki/Levocarnitine" class="mw-redirect" title="Levocarnitine">Levocarnitine</a></li> <li><a href="/wiki/Mercaptamine" class="mw-redirect" title="Mercaptamine">Mercaptamine</a></li> <li><a href="/wiki/Metreleptin" title="Metreleptin">Metreleptin</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Enzyme" title="Enzyme">Enzymes</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Amino_acid" title="Amino acid">Amino acids</a>: <i><a href="/wiki/Phenylalanine_ammonia-lyase" title="Phenylalanine ammonia-lyase">phenylalanine ammonia-lyase</a></i> (<a href="/wiki/Pegvaliase" title="Pegvaliase">Pegvaliase</a>)</li></ul> <ul><li><a href="/wiki/Carbohydrate_metabolism" title="Carbohydrate metabolism">Carbohydrate metabolism</a>: <i><a href="/wiki/Sucrase" title="Sucrase">sucrase</a></i> (<a href="/wiki/Sacrosidase" title="Sacrosidase">Sacrosidase</a>)</li> <li><i><a href="/wiki/Alpha-glucosidase" class="mw-redirect" title="Alpha-glucosidase">alpha-glucosidase</a></i> (<a href="/wiki/Alglucosidase_alfa" title="Alglucosidase alfa">Alglucosidase alfa</a>, <a href="/wiki/Avalglucosidase_alfa" title="Avalglucosidase alfa">Avalglucosidase alfa</a>, <a href="/wiki/Cipaglucosidase_alfa" title="Cipaglucosidase alfa">Cipaglucosidase alfa</a>)</li></ul> <ul><li><a href="/wiki/Glycolipid" title="Glycolipid">Glycolipid</a>/<a href="/wiki/Sphingolipid" title="Sphingolipid">sphingolipid</a>: <i><a href="/wiki/Glucocerebrosidase" title="Glucocerebrosidase">glucocerebrosidase</a></i> (<a href="/wiki/Alglucerase" title="Alglucerase">Alglucerase</a></li> <li><a href="/wiki/Imiglucerase" title="Imiglucerase">Imiglucerase</a></li> <li><a href="/wiki/Taliglucerase_alfa" title="Taliglucerase alfa">Taliglucerase alfa</a></li> <li><a href="/wiki/Velaglucerase_alfa" title="Velaglucerase alfa">Velaglucerase alfa</a>)</li> <li><i><a href="/wiki/Alpha-galactosidase" class="mw-redirect" title="Alpha-galactosidase">alpha-galactosidase</a></i> (<a href="/wiki/Agalsidase_alfa" class="mw-redirect" title="Agalsidase alfa">Agalsidase alfa</a></li> <li><a href="/wiki/Agalsidase_beta" class="mw-redirect" title="Agalsidase beta">Agalsidase beta</a></li> <li><a href="/wiki/Pegunigalsidase_alfa" title="Pegunigalsidase alfa">Pegunigalsidase alfa</a>)</li></ul> <ul><li><a href="/wiki/Glycosaminoglycan" title="Glycosaminoglycan">Glycosaminoglycan</a>: <i><a href="/wiki/Iduronidase" title="Iduronidase">iduronidase</a></i> (<a href="/wiki/Laronidase" class="mw-redirect" title="Laronidase">Laronidase</a>)</li> <li><i><a href="/wiki/Arylsulfatase_B" title="Arylsulfatase B">arylsulfatase B</a></i> (<a href="/wiki/Galsulfase" class="mw-redirect" title="Galsulfase">Galsulfase</a>)</li> <li><i><a href="/wiki/Iduronate-2-sulfatase" title="Iduronate-2-sulfatase">iduronate-2-sulfatase</a></i> (<a href="/wiki/Idursulfase" title="Idursulfase">Idursulfase</a>)</li> <li><a href="/w/index.php?title=Idursulfase_beta&action=edit&redlink=1" class="new" title="Idursulfase beta (page does not exist)">idursulfase beta</a></li></ul> <ul><li><a href="/wiki/Lipid" title="Lipid">Lipid</a>: <i><a href="/wiki/Lysosomal_lipase" title="Lysosomal lipase">lysosomal lipase</a></i> (<a href="/wiki/Sebelipase_alfa" title="Sebelipase alfa">Sebelipase alfa</a>)</li></ul> <ul><li><a href="/wiki/Phosphate" title="Phosphate">Phosphate</a>: <a href="/wiki/Asfotase_alfa" title="Asfotase alfa">Asfotase alfa</a></li></ul> <ul><li><a href="/wiki/Atidarsagene_autotemcel" title="Atidarsagene autotemcel">atidarsagene autotemcel</a></li> <li><a href="/wiki/Cerliponase_alfa" title="Cerliponase alfa">cerliponase alfa</a></li> <li><a href="/wiki/Eladocagene_exuparvovec" title="Eladocagene exuparvovec">eladocagene exuparvovec</a></li> <li><a href="/wiki/Elosulfase_alfa" title="Elosulfase alfa">elosulfase alfa</a></li> <li><a href="/wiki/Olipudase_alfa" title="Olipudase alfa">olipudase alfa</a></li> <li><a href="/w/index.php?title=Pabinafusp_alfa&action=edit&redlink=1" class="new" title="Pabinafusp alfa (page does not exist)">pabinafusp alfa</a></li> <li><a href="/wiki/Pegzilarginase" title="Pegzilarginase">pegzilarginase</a></li> <li><a href="/wiki/Vestronidase_alfa" title="Vestronidase alfa">vestronidase alfa</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Anethole_trithione" title="Anethole trithione">Anethole trithione</a></li> <li><a href="/wiki/Eliglustat" title="Eliglustat">Eliglustat</a></li> <li><a href="/wiki/Givosiran" title="Givosiran">Givosiran</a></li> <li><a href="/wiki/Glycerol_phenylbutyrate" title="Glycerol phenylbutyrate">Glycerol phenylbutyrate</a></li> <li><a href="/wiki/Leriglitazone" title="Leriglitazone">Leriglitazone</a></li> <li><a href="/wiki/Lumasiran" title="Lumasiran">Lumasiran</a></li> <li><a href="/wiki/Miglustat" title="Miglustat">Miglustat</a></li> <li><a href="/wiki/Nedosiran" title="Nedosiran">Nedosiran</a></li> <li><a href="/wiki/Nitisinone" title="Nitisinone">Nitisinone</a></li> <li><a class="mw-selflink selflink">Sapropterin</a></li> <li><a href="/wiki/Sepiapterin" title="Sepiapterin">Sepiapterin</a></li> <li><a href="/wiki/Sodium_benzoate" title="Sodium benzoate">Sodium benzoate</a> (<a href="/wiki/Sodium_phenylacetate/sodium_benzoate" title="Sodium phenylacetate/sodium benzoate">+sodium phenylacetate</a>)</li> <li><a href="/wiki/Sodium_phenylbutyrate" title="Sodium phenylbutyrate">Sodium phenylbutyrate</a></li> <li><a href="/wiki/Teduglutide" title="Teduglutide">Teduglutide</a></li> <li><a href="/wiki/Trientine" class="mw-redirect" title="Trientine">Trientine</a></li> <li><a href="/wiki/Tioctic_acid" class="mw-redirect" title="Tioctic acid">Tioctic acid</a></li> <li><a href="/wiki/Triheptanoin" title="Triheptanoin">Triheptanoin</a></li> <li><a href="/wiki/Uridine_triacetate" title="Uridine triacetate">Uridine triacetate</a></li> <li><a href="/wiki/Velmanase_alfa" title="Velmanase alfa">Velmanase alfa</a></li> <li><a href="/wiki/Zinc_acetate" title="Zinc acetate">Zinc acetate</a></li></ul> </div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374" /><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235" /></div><div role="navigation" class="navbox" aria-labelledby="Nitric_oxide_signaling_modulators330" style="padding:3px"><table class="nowraplinks mw-collapsible mw-collapsed navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374" /><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1239400231" /><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Nitric_oxide_signaling_modulators" title="Template:Nitric oxide signaling modulators"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Nitric_oxide_signaling_modulators" title="Template talk:Nitric oxide signaling modulators"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Nitric_oxide_signaling_modulators" title="Special:EditPage/Template:Nitric oxide signaling modulators"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Nitric_oxide_signaling_modulators330" style="font-size:114%;margin:0 4em"><a href="/wiki/Nitric_oxide" title="Nitric oxide">Nitric oxide</a> <a href="/wiki/Cell_signaling" title="Cell signaling">signaling</a> <a href="/wiki/Receptor_modulator" title="Receptor modulator">modulators</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Nitric_oxide" title="Nitric oxide">Forms</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Nitroxyl" title="Nitroxyl">Nitroxyl anion (NO<sup>−</sup>; oxonitrate(1-), hyponitrite anion)</a></li> <li><a href="/wiki/Nitric_oxide" title="Nitric oxide">Nitric oxide (NO<sup>⋅</sup>; nitrogen monoxide)</a></li> <li><a href="/wiki/Nitrosonium" title="Nitrosonium">Nitrosonium (NO<sup>+</sup>; nitrosyl cation)</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Biological_target" title="Biological target">Targets</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="sGC33" scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Soluble_guanylate_cyclase" class="mw-redirect" title="Soluble guanylate cyclase">sGC</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i>Activators/stimulators:</i> <a href="/w/index.php?title=Ataciguat&action=edit&redlink=1" class="new" title="Ataciguat (page does not exist)">Ataciguat</a></li> <li><a href="/w/index.php?title=BAY_41-2272&action=edit&redlink=1" class="new" title="BAY 41-2272 (page does not exist)">BAY 41-2272</a></li> <li><a href="/w/index.php?title=BAY_41-8543&action=edit&redlink=1" class="new" title="BAY 41-8543 (page does not exist)">BAY 41-8543</a></li> <li><a href="/w/index.php?title=BAY_60-4552&action=edit&redlink=1" class="new" title="BAY 60-4552 (page does not exist)">BAY 60-4552</a></li> <li><a href="/w/index.php?title=BI-703704&action=edit&redlink=1" class="new" title="BI-703704 (page does not exist)">BI-703704</a></li> <li><a href="/wiki/Cinaciguat" title="Cinaciguat">Cinaciguat (BAY 58-2667)</a></li> <li><a href="/w/index.php?title=GSK-2181236A&action=edit&redlink=1" class="new" title="GSK-2181236A (page does not exist)">GSK-2181236A</a></li> <li><a href="/w/index.php?title=Praliciguat&action=edit&redlink=1" class="new" title="Praliciguat (page does not exist)">Praliciguat</a></li> <li><a href="/wiki/Riociguat" title="Riociguat">Riociguat</a></li> <li><a href="/wiki/Vericiguat" title="Vericiguat">Vericiguat</a></li></ul> <ul><li><i>Inhibitors:</i> <a href="/w/index.php?title=1H-(1,2,4)Oxadiazolo(4,3-a)quinoxalin-1-one&action=edit&redlink=1" class="new" title="1H-(1,2,4)Oxadiazolo(4,3-a)quinoxalin-1-one (page does not exist)">ODQ</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/NO_donor" class="mw-redirect" title="NO donor">NO donors</a><br /><small>(<a href="/wiki/Prodrug" title="Prodrug">prodrugs</a>)</small></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/Nitrate" title="Nitrate">Nitrates</a>:</i> <a href="/wiki/Diethylene_glycol_dinitrate" title="Diethylene glycol dinitrate">Diethylene glycol dinitrate (DEGDN)</a></li> <li><a href="/wiki/Erythritol_tetranitrate" title="Erythritol tetranitrate">Erythritol tetranitrate (ETN)</a></li> <li><a href="/wiki/Ethylene_glycol_dinitrate" title="Ethylene glycol dinitrate">Ethylene glycol dinitrate (EGDN; nitroglycol)</a></li> <li><a href="/wiki/Isosorbide_mononitrate" title="Isosorbide mononitrate">Isosorbide mononitrate (ISMN)</a></li> <li><a href="/wiki/Isosorbide_dinitrate" title="Isosorbide dinitrate">Isosorbide dinitrate (ISDN)</a></li> <li><a href="/wiki/Itramin_tosilate" title="Itramin tosilate">Itramin tosilate</a></li> <li><a href="/wiki/Mannitol_hexanitrate" title="Mannitol hexanitrate">Mannitol hexanitrate</a></li> <li><a href="/wiki/Naproxcinod" title="Naproxcinod">Naproxcinod (nitronaproxen; AZD-3582, HCT-3012)</a></li> <li><a href="/w/index.php?title=NCX-466&action=edit&redlink=1" class="new" title="NCX-466 (page does not exist)">NCX-466</a></li> <li><a href="/w/index.php?title=NCX-2216&action=edit&redlink=1" class="new" title="NCX-2216 (page does not exist)">NCX-2216</a></li> <li><a href="/w/index.php?title=NCX-4016&action=edit&redlink=1" class="new" title="NCX-4016 (page does not exist)">NCX-4016</a></li> <li><a href="/w/index.php?title=NCX_4040&action=edit&redlink=1" class="new" title="NCX 4040 (page does not exist)">NCX 4040</a></li> <li><a href="/w/index.php?title=NCX-4215&action=edit&redlink=1" class="new" title="NCX-4215 (page does not exist)">NCX-4215</a></li> <li><a href="/wiki/Nicorandil" title="Nicorandil">Nicorandil</a></li> <li><a href="/wiki/Nipradilol" title="Nipradilol">Nipradilol (K-351)</a></li> <li><a href="/wiki/Nitrate" title="Nitrate">Nitrate (NO<span class="nowrap"><span style="display:inline-block;margin-bottom:-0.3em;vertical-align:-0.4em;line-height:1.2em;font-size:80%;text-align:left"><sup style="font-size:inherit;line-height:inherit;vertical-align:baseline">−</sup><br /><sub style="font-size:inherit;line-height:inherit;vertical-align:baseline">3</sub></span></span>)</a></li> <li><a href="/w/index.php?title=Nitroatorvastatin&action=edit&redlink=1" class="new" title="Nitroatorvastatin (page does not exist)">Nitroatorvastatin (NCX-6560)</a></li> <li><a href="/w/index.php?title=Nitroflurbiprofen&action=edit&redlink=1" class="new" title="Nitroflurbiprofen (page does not exist)">Nitroflurbiprofen (HCT-1026)</a></li> <li><a href="/w/index.php?title=Nitrofluvastatin&action=edit&redlink=1" class="new" title="Nitrofluvastatin (page does not exist)">Nitrofluvastatin</a></li> <li><a href="/wiki/Nitroglycerin_(drug)" class="mw-redirect" title="Nitroglycerin (drug)">Nitroglycerin (glyceryl trinitrate (GTN))</a></li> <li><a href="/w/index.php?title=Nitropravastatin&action=edit&redlink=1" class="new" title="Nitropravastatin (page does not exist)">Nitropravastatin (NCX-6550)</a></li> <li><a href="/wiki/Pentaerithrityl_tetranitrate" class="mw-redirect" title="Pentaerithrityl tetranitrate">Pentaerithrityl tetranitrate (PETN)</a></li> <li><a href="/wiki/Propatylnitrate" title="Propatylnitrate">Propatylnitrate</a></li> <li><a href="/wiki/Propylene_glycol_dinitrate" title="Propylene glycol dinitrate">Propylene glycol dinitrate (PGDN)</a></li> <li><a href="/w/index.php?title=Sodium_trioxodinitrate&action=edit&redlink=1" class="new" title="Sodium trioxodinitrate (page does not exist)">Sodium trioxodinitrate (Angeli's salt)</a></li> <li>Tenitramine</li> <li><a href="/wiki/Trolnitrate" title="Trolnitrate">Trolnitrate</a></li></ul> <ul><li><i><a href="/wiki/Nitroso_compound" class="mw-redirect" title="Nitroso compound">Nitroso compounds</a>/<a href="/wiki/Nitrite" title="Nitrite">nitrites</a>:</i> <a href="/wiki/Nitrite" title="Nitrite">Nitrite (NO<span class="nowrap"><span style="display:inline-block;margin-bottom:-0.3em;vertical-align:-0.4em;line-height:1.2em;font-size:80%;text-align:left"><sup style="font-size:inherit;line-height:inherit;vertical-align:baseline">−</sup><br /><sub style="font-size:inherit;line-height:inherit;vertical-align:baseline">2</sub></span></span>)</a>; <i>O-Nitroso compounds (<a href="/wiki/Alkyl_nitrite" title="Alkyl nitrite">alkyl nitrites</a>):</i> <a href="/wiki/Amyl_nitrite" title="Amyl nitrite">Amyl nitrite (isoamyl nitrite, isopentyl nitrite)</a></li> <li><a href="/wiki/Cyclohexyl_nitrite" title="Cyclohexyl nitrite">Cyclohexyl nitrite</a></li> <li><a href="/wiki/Ethyl_nitrite" title="Ethyl nitrite">Ethyl nitrite</a></li> <li><a href="/wiki/Hexyl_nitrite" title="Hexyl nitrite">Hexyl nitrite</a></li> <li><a href="/wiki/Isobutyl_nitrite" title="Isobutyl nitrite">Isobutyl nitrite (2-methylpropyl nitrite)</a></li> <li><a href="/wiki/Isopropyl_nitrite" title="Isopropyl nitrite">Isopropyl nitrite</a></li> <li><a href="/wiki/Methyl_nitrite" title="Methyl nitrite">Methyl nitrite</a></li> <li><a href="/wiki/Butyl_nitrite" title="Butyl nitrite"><i>n</i>-Butyl nitrite</a></li> <li><a href="/wiki/Pentyl_nitrite" title="Pentyl nitrite">Pentyl nitrite</a></li> <li><a href="/wiki/Tert-Butyl_nitrite" title="Tert-Butyl nitrite"><i>tert</i>-Butyl nitrite</a>; <i><a href="/wiki/S-Nitrosothiol" title="S-Nitrosothiol">S-Nitroso compounds</a> (thionitrites):</i> <a href="/w/index.php?title=LA810&action=edit&redlink=1" class="new" title="LA810 (page does not exist)">LA810</a></li> <li><a href="/w/index.php?title=S-Nitrosoalbumin&action=edit&redlink=1" class="new" title="S-Nitrosoalbumin (page does not exist)">S-Nitrosoalbumin (SNALB)</a></li> <li><a href="/w/index.php?title=S-Nitrosated_AR545C&action=edit&redlink=1" class="new" title="S-Nitrosated AR545C (page does not exist)">S-Nitrosated AR545C</a></li> <li><a href="/w/index.php?title=S-Nitroso-N-acetylcysteine&action=edit&redlink=1" class="new" title="S-Nitroso-N-acetylcysteine (page does not exist)">S-Nitroso-N-acetylcysteine (SNAC)</a></li> <li><a href="/wiki/S-Nitroso-N-acetylpenicillamine" title="S-Nitroso-N-acetylpenicillamine">S-Nitroso-N-acetylpenicillamine (SNAP)</a></li> <li><a href="/w/index.php?title=S-Nitroso-N-valerylpenicillamine&action=edit&redlink=1" class="new" title="S-Nitroso-N-valerylpenicillamine (page does not exist)">S-Nitroso-N-valerylpenicillamine (SNVP)</a></li> <li><a href="/w/index.php?title=S-Nitrosocaptopril&action=edit&redlink=1" class="new" title="S-Nitrosocaptopril (page does not exist)">S-Nitrosocaptopril (SNO-Cap)</a></li> <li><a href="/w/index.php?title=S-Nitrosocysteine&action=edit&redlink=1" class="new" title="S-Nitrosocysteine (page does not exist)">S-Nitrosocysteine (SNC, CysNO, SNO-Cys)</a></li> <li><a href="/w/index.php?title=S-Nitrosodiclofenac&action=edit&redlink=1" class="new" title="S-Nitrosodiclofenac (page does not exist)">S-Nitrosodiclofenac</a></li> <li><a href="/wiki/S-Nitrosoglutathione" title="S-Nitrosoglutathione">S-Nitrosoglutathione (GSNO, SNOG)</a></li> <li><a href="/w/index.php?title=S-Nitrosated_tissue-type_plasminogen_activator&action=edit&redlink=1" class="new" title="S-Nitrosated tissue-type plasminogen activator (page does not exist)">SNO-t-PA</a></li> <li><a href="/w/index.php?title=S-Nitrosated_von_Willebrand_factor&action=edit&redlink=1" class="new" title="S-Nitrosated von Willebrand factor (page does not exist)">SNO-vWF</a>; <i>N-Nitroso compounds (e.g., <a href="/wiki/Nitrosamine" title="Nitrosamine">nitrosamines</a>):</i> <a href="/w/index.php?title=SIN-1A&action=edit&redlink=1" class="new" title="SIN-1A (page does not exist)">SIN-1A</a></li></ul> <ul><li><i>Nitrosyl compounds:</i> <i><a href="/wiki/Metal_nitrosyl_complex" title="Metal nitrosyl complex">Metal nitrosyl complexes</a>:</i> <a href="/wiki/Roussin%27s_black_salt" title="Roussin's black salt">Roussin's black salt</a></li> <li><a href="/wiki/Roussin%27s_red_salt" title="Roussin's red salt">Roussin's red salt</a></li> <li><a href="/wiki/Sodium_nitroprusside" title="Sodium nitroprusside">Sodium nitroprusside (SNP)</a></li></ul> <ul><li><i><a href="/wiki/NONOate" title="NONOate">NONOates</a> (diazeniumdiolates):</i> <a href="/w/index.php?title=Diethylamine/NO&action=edit&redlink=1" class="new" title="Diethylamine/NO (page does not exist)">Diethylamine/NO (DEA/NO)</a></li> <li><a href="/w/index.php?title=Diethylenetriamine/NO&action=edit&redlink=1" class="new" title="Diethylenetriamine/NO (page does not exist)">Diethylenetriamine/NO (DETA/NO)</a></li> <li><a href="/w/index.php?title=GLO/NO&action=edit&redlink=1" class="new" title="GLO/NO (page does not exist)">GLO/NO</a></li> <li><a href="/w/index.php?title=JS-K&action=edit&redlink=1" class="new" title="JS-K (page does not exist)">JS-K</a></li> <li><a href="/w/index.php?title=Methylamine_hexamethylene_methylamine/NO&action=edit&redlink=1" class="new" title="Methylamine hexamethylene methylamine/NO (page does not exist)">Methylamine hexamethylene methylamine/NO (MAHMA/NO)</a></li> <li><a href="/w/index.php?title=PROLI/NO&action=edit&redlink=1" class="new" title="PROLI/NO (page does not exist)">PROLI/NO</a></li> <li><a href="/w/index.php?title=Spermine/NO&action=edit&redlink=1" class="new" title="Spermine/NO (page does not exist)">Spermine/NO (SPER/NO)</a></li> <li><a href="/w/index.php?title=V-PYRRO/NO&action=edit&redlink=1" class="new" title="V-PYRRO/NO (page does not exist)">V-PYRRO/NO</a></li></ul> <ul><li><i>Heterocyclic compounds:</i> <i><a href="/wiki/Furoxan" title="Furoxan">Furoxans</a>:</i> <a href="/wiki/Furoxan" title="Furoxan">Furoxan</a></li> <li><a href="/w/index.php?title=REC15/2739&action=edit&redlink=1" class="new" title="REC15/2739 (page does not exist)">REC15/2739</a>; <i><a href="/w/index.php?title=Sydnonimine&action=edit&redlink=1" class="new" title="Sydnonimine (page does not exist)">Sydnonimines</a>:</i> <a href="/wiki/Feprosidnine" title="Feprosidnine">Feprosidnine</a></li> <li><a href="/wiki/Linsidomine" title="Linsidomine">Linsidomine (SIN-1)</a></li> <li><a href="/wiki/Molsidomine" title="Molsidomine">Molsidomine (SIN-10)</a></li> <li><a href="/w/index.php?title=Sydnonimine&action=edit&redlink=1" class="new" title="Sydnonimine (page does not exist)">Sydnonimine</a></li></ul> <ul><li><i>Unsorted:</i> <a href="/wiki/Cimlanod" title="Cimlanod">Cimlanod</a></li> <li><a href="/w/index.php?title=FK-409&action=edit&redlink=1" class="new" title="FK-409 (page does not exist)">FK-409</a></li> <li><a href="/w/index.php?title=FR144220&action=edit&redlink=1" class="new" title="FR144220 (page does not exist)">FR144220</a></li> <li><a href="/w/index.php?title=FR146881&action=edit&redlink=1" class="new" title="FR146881 (page does not exist)">FR146881</a></li> <li><a href="/w/index.php?title=N-Acetyl-N-acetoxy-4-chlorobenzenesulfonamide&action=edit&redlink=1" class="new" title="N-Acetyl-N-acetoxy-4-chlorobenzenesulfonamide (page does not exist)">N-Acetyl-N-acetoxy-4-chlorobenzenesulfonamide</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Enzyme" title="Enzyme">Enzyme</a><br /><small>(<a href="/wiki/Enzyme_inhibitor" title="Enzyme inhibitor">inhibitors</a>)</small></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Nitric_oxide_synthase" title="Nitric oxide synthase">NOS</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/NOS1" title="NOS1">nNOS</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/w/index.php?title=3-Bromo-7-nitroindazole&action=edit&redlink=1" class="new" title="3-Bromo-7-nitroindazole (page does not exist)">3-Bromo-7-nitroindazole</a></li> <li><a href="/w/index.php?title=3-Chloroindazole&action=edit&redlink=1" class="new" title="3-Chloroindazole (page does not exist)">3-Chloroindazole</a></li> <li><a href="/w/index.php?title=3-Chloro-5-nitroindazole&action=edit&redlink=1" class="new" title="3-Chloro-5-nitroindazole (page does not exist)">3-Chloro-5-nitroindazole</a></li> <li><a href="/w/index.php?title=5-Nitroindazole&action=edit&redlink=1" class="new" title="5-Nitroindazole (page does not exist)">5-Nitroindazole</a></li> <li><a href="/w/index.php?title=6-Nitroindazole&action=edit&redlink=1" class="new" title="6-Nitroindazole (page does not exist)">6-Nitroindazole</a></li> <li><a href="/wiki/7-Nitroindazole" title="7-Nitroindazole">7-Nitroindazole</a></li> <li><a href="/w/index.php?title=A-84643&action=edit&redlink=1" class="new" title="A-84643 (page does not exist)">A-84643</a></li> <li><a href="/wiki/Aminoguanidine" class="mw-redirect" title="Aminoguanidine">Aminoguanidine (pimagedine)</a></li> <li><a href="/w/index.php?title=ARL-17477&action=edit&redlink=1" class="new" title="ARL-17477 (page does not exist)">ARL-17477</a></li> <li><a href="/wiki/Indazole" title="Indazole">Indazole</a></li> <li><a href="/w/index.php?title=N5-(1-Iminoethyl)-L-ornithine&action=edit&redlink=1" class="new" title="N5-(1-Iminoethyl)-L-ornithine (page does not exist)">N<sup>5</sup>-(1-Iminoethyl)-L-ornithine (<small>L</small>-NIO)</a></li> <li><a href="/w/index.php?title=N%CF%89-Methyl-L-arginine&action=edit&redlink=1" class="new" title="Nω-Methyl-L-arginine (page does not exist)">N<sup>ω</sup>-Methyl-<small>L</small>-arginine (<small>L</small>-NMA)</a></li> <li><a href="/w/index.php?title=N%CF%89-Propyl-L-arginine&action=edit&redlink=1" class="new" title="Nω-Propyl-L-arginine (page does not exist)">N<sup>ω</sup>-Propyl-<small>L</small>-arginine (<small>L</small>-NPA)</a></li> <li><a href="/wiki/Nitroarginine" title="Nitroarginine">Nitroarginine (NNA, NOARG)</a></li> <li><a href="/wiki/Pentamidine_isethionate" class="mw-redirect" title="Pentamidine isethionate">Pentamidine isethionate</a></li> <li><a href="/w/index.php?title=1-(2-Trifluoromethylphenyl)imidazole&action=edit&redlink=1" class="new" title="1-(2-Trifluoromethylphenyl)imidazole (page does not exist)">TRIM</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Nitric_oxide_synthase_2_(inducible)" title="Nitric oxide synthase 2 (inducible)">iNOS</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/w/index.php?title=1-Amino-2-hydroxyguanidine&action=edit&redlink=1" class="new" title="1-Amino-2-hydroxyguanidine (page does not exist)">1-Amino-2-hydroxyguanidine</a></li> <li><a href="/w/index.php?title=2-Ethylaminoguanidine&action=edit&redlink=1" class="new" title="2-Ethylaminoguanidine (page does not exist)">2-Ethylaminoguanidine</a></li> <li><a href="/w/index.php?title=2-Iminopiperidine&action=edit&redlink=1" class="new" title="2-Iminopiperidine (page does not exist)">2-Iminopiperidine</a></li> <li><a href="/w/index.php?title=1400W&action=edit&redlink=1" class="new" title="1400W (page does not exist)">1400W</a></li> <li><a href="/w/index.php?title=S-aminoethylisothiourea&action=edit&redlink=1" class="new" title="S-aminoethylisothiourea (page does not exist)">AEITU</a></li> <li><a href="/wiki/Aminoguanidine" class="mw-redirect" title="Aminoguanidine">Aminoguanidine (pimagedine)</a></li> <li><a href="/w/index.php?title=2-Amino-5,6-dihydro-6-methyl-4H-1,3-thiazine&action=edit&redlink=1" class="new" title="2-Amino-5,6-dihydro-6-methyl-4H-1,3-thiazine (page does not exist)">AMT</a></li> <li><a href="/w/index.php?title=AR-C_102222&action=edit&redlink=1" class="new" title="AR-C 102222 (page does not exist)">AR-C 102222</a></li> <li><a href="/w/index.php?title=BYK-191023&action=edit&redlink=1" class="new" title="BYK-191023 (page does not exist)">BYK-191023</a></li> <li><a href="/wiki/Canavanine" title="Canavanine">Canavanine</a></li> <li><a href="/w/index.php?title=Cindunistat&action=edit&redlink=1" class="new" title="Cindunistat (page does not exist)">Cindunistat (SD-6010)</a></li> <li><a href="/w/index.php?title=S-Ethylisothiourea&action=edit&redlink=1" class="new" title="S-Ethylisothiourea (page does not exist)">EITU</a></li> <li><a href="/w/index.php?title=S-Isopropylisothiourea&action=edit&redlink=1" class="new" title="S-Isopropylisothiourea (page does not exist)">IPTU</a></li> <li><a href="/w/index.php?title=S-Methylisothiourea&action=edit&redlink=1" class="new" title="S-Methylisothiourea (page does not exist)">MITU</a></li> <li><a href="/w/index.php?title=N5-(1-Iminoethyl)-L-ornithine&action=edit&redlink=1" class="new" title="N5-(1-Iminoethyl)-L-ornithine (page does not exist)">N<sup>5</sup>-(1-Iminoethyl)-<small>L</small>-ornithine (<small>L</small>-NIO)</a></li> <li><a href="/w/index.php?title=N6-(1-Iminoethyl)-L-lysine&action=edit&redlink=1" class="new" title="N6-(1-Iminoethyl)-L-lysine (page does not exist)">N<sup>6</sup>-(1-Iminoethyl)-<small>L</small>-lysine (<small>L</small>-NIL)</a></li> <li><a href="/w/index.php?title=N%CF%89-Methyl-L-arginine&action=edit&redlink=1" class="new" title="Nω-Methyl-L-arginine (page does not exist)">N<sup>ω</sup>-Methyl-<small>L</small>-arginine (<small>L</small>-NMA)</a></li> <li><a href="/w/index.php?title=Ronopterin&action=edit&redlink=1" class="new" title="Ronopterin (page does not exist)">Ronopterin (VAS-203)</a></li> <li><a href="/w/index.php?title=1-(2-Trifluoromethylphenyl)imidazole&action=edit&redlink=1" class="new" title="1-(2-Trifluoromethylphenyl)imidazole (page does not exist)">TRIM</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Endothelial_NOS" title="Endothelial NOS">eNOS</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Aminoguanidine" class="mw-redirect" title="Aminoguanidine">Aminoguanidine (pimagedine)</a></li> <li><a href="/w/index.php?title=N5-(1-Iminoethyl)-L-ornithine&action=edit&redlink=1" class="new" title="N5-(1-Iminoethyl)-L-ornithine (page does not exist)">N<sup>5</sup>-(1-Iminoethyl)-<small>L</small>-ornithine (<small>L</small>-NIO)</a></li> <li><a href="/w/index.php?title=N%CF%89-Methyl-L-arginine&action=edit&redlink=1" class="new" title="Nω-Methyl-L-arginine (page does not exist)">N<sup>ω</sup>-Methyl-<small>L</small>-arginine (<small>L</small>-NMA)</a></li> <li><a href="/wiki/Nitroarginine" title="Nitroarginine">Nitroarginine (NNA, NOARG)</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;">Unsorted</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Asymmetric_dimethylarginine" title="Asymmetric dimethylarginine">Asymmetric dimethylarginine (ADMA)</a></li> <li><a href="/w/index.php?title=CKD-712&action=edit&redlink=1" class="new" title="CKD-712 (page does not exist)">CKD-712</a></li> <li><a href="/w/index.php?title=Guanidinoethyldisulfide&action=edit&redlink=1" class="new" title="Guanidinoethyldisulfide (page does not exist)">Guanidinoethyldisulfide (GED)</a></li> <li><a href="/w/index.php?title=GW-273629&action=edit&redlink=1" class="new" title="GW-273629 (page does not exist)">GW-273629</a></li> <li><a href="/wiki/Indospicine" title="Indospicine">Indospicine</a></li> <li><a href="/w/index.php?title=KD-7040&action=edit&redlink=1" class="new" title="KD-7040 (page does not exist)">KD-7040</a></li> <li><a href="/w/index.php?title=Nitroarginine_methyl_ester&action=edit&redlink=1" class="new" title="Nitroarginine methyl ester (page does not exist)">Nitroarginine methyl ester (NAME)</a></li> <li><a href="/w/index.php?title=NCX-456&action=edit&redlink=1" class="new" title="NCX-456 (page does not exist)">NCX-456</a></li> <li><a href="/w/index.php?title=NXN-462&action=edit&redlink=1" class="new" title="NXN-462 (page does not exist)">NXN-462</a></li> <li><a href="/w/index.php?title=ONO-1714&action=edit&redlink=1" class="new" title="ONO-1714 (page does not exist)">ONO-1714</a></li> <li><a href="/w/index.php?title=VAS-2381&action=edit&redlink=1" class="new" title="VAS-2381 (page does not exist)">VAS-2381</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/Arginase" title="Arginase">Arginase</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/w/index.php?title=2S-Amino-6-boronohexonic_acid&action=edit&redlink=1" class="new" title="2S-Amino-6-boronohexonic acid (page does not exist)">ABH</a></li> <li><a href="/w/index.php?title=N%CF%89-Hydroxy-L-arginine&action=edit&redlink=1" class="new" title="Nω-Hydroxy-L-arginine (page does not exist)">N<sup>ω</sup>-Hydroxy-<small>L</small>-arginine (NOHA)</a></li> <li><a href="/wiki/Chlorogenic_acid" title="Chlorogenic acid">chlorogenic acid</a></li> <li><a href="/wiki/Ginseng" title="Ginseng">ginseng</a></li> <li><a href="/wiki/Epicatechin" class="mw-redirect" title="Epicatechin">epicatechin</a></li> <li><a href="/wiki/Ornithine" title="Ornithine">ornithine</a></li> <li><a href="/wiki/Norvaline" title="Norvaline">norvaline</a></li> <li><a href="/wiki/Lysine" title="Lysine">lysine</a></li> <li><a href="/w/index.php?title=Alpha_aminoacids&action=edit&redlink=1" class="new" title="Alpha aminoacids (page does not exist)">alpha aminoacids</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;"><a href="/wiki/CAMK" title="CAMK">CAMK</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/w/index.php?title=Calmidazolium&action=edit&redlink=1" class="new" title="Calmidazolium (page does not exist)">Calmidazolium</a></li> <li><a href="/w/index.php?title=W-7_(drug)&action=edit&redlink=1" class="new" title="W-7 (drug) (page does not exist)">W-7</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;text-align:center;">Others</th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i>Precursors:</i> <a href="/wiki/Arginine" title="Arginine"><small>L</small>-Arginine</a></li> <li><a href="/w/index.php?title=N%CF%89-Hydroxy-L-arginine&action=edit&redlink=1" class="new" title="Nω-Hydroxy-L-arginine (page does not exist)">N<sup>ω</sup>-Hydroxy-<small>L</small>-arginine (NOHA)</a></li></ul> <ul><li><i>Cofactors:</i> <a href="/wiki/Nicotinamide_adenine_dinucleotide_phosphate" title="Nicotinamide adenine dinucleotide phosphate">NADPH</a></li> <li><a href="/wiki/Flavin_adenine_dinucleotide" title="Flavin adenine dinucleotide">FAD</a></li> <li><a href="/wiki/Flavin_mononucleotide" title="Flavin mononucleotide">FMN</a></li> <li><a href="/wiki/Heme" title="Heme">Heme</a></li> <li><a class="mw-selflink selflink">BH<sub>4</sub></a></li> <li><a href="/wiki/Calmodulin" title="Calmodulin">CaM</a></li> <li><a href="/wiki/Oxygen" title="Oxygen">O<sub>2</sub></a></li> <li><a href="/wiki/Calcium" title="Calcium">Ca<sup>2+</sup></a></li></ul> <ul><li><i>Indirect/downstream NO modulators:</i> <a href="/wiki/ACE_inhibitor" title="ACE inhibitor">ACE inhibitors</a>/<a href="/wiki/Angiotensin_II_receptor_antagonist" class="mw-redirect" title="Angiotensin II receptor antagonist">AT-II receptor antagonists</a> (e.g., <a href="/wiki/Captopril" title="Captopril">captopril</a>, <a href="/wiki/Losartan" title="Losartan">losartan</a>)</li> <li><a href="/wiki/Endothelin_receptor_antagonist" title="Endothelin receptor antagonist">ET<sub>B</sub> receptor antagonists</a> (e.g., <a href="/wiki/Bosentan" title="Bosentan">bosentan</a>)</li> <li><a href="/wiki/L-Type_calcium_channel" class="mw-redirect" title="L-Type calcium channel">L-Type calcium channel</a> <a href="/wiki/Calcium_channel_blocker" title="Calcium channel blocker">blockers</a> (e.g., <a href="/wiki/Dihydropyridine" class="mw-redirect" title="Dihydropyridine">dihydropyridines</a>: <a href="/wiki/Nifedipine" title="Nifedipine">nifedipine</a>)</li> <li><a href="/wiki/Nebivolol" title="Nebivolol">Nebivolol</a> (<a href="/wiki/Beta_blocker" title="Beta blocker">beta blocker</a>)</li> <li><a href="/wiki/PDE5_inhibitor" title="PDE5 inhibitor">PDE5 inhibitors</a> (e.g., <a href="/wiki/Sildenafil" title="Sildenafil">sildenafil</a>)</li> <li>non-selective PDE inhibitors (e.g., <a href="/wiki/Caffeine" title="Caffeine">caffeine</a>)</li> <li>PDE9 inhibitors (e.g., <a href="/wiki/Paraxanthine" title="Paraxanthine">paraxanthine</a>)</li> <li>cGMP 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