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MT-TI - Wikipedia

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<div class="mw-indicators"> </div> <div id="siteSub" class="noprint">From Wikipedia, the free encyclopedia</div> </div> <div id="contentSub"><div id="mw-content-subtitle"></div></div> <div id="mw-content-text" class="mw-body-content"><div class="mw-content-ltr mw-parser-output" lang="en" dir="ltr"><div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Transfer RNA</div> <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"><tbody><tr><th colspan="2" class="infobox-above">mitochondrially encoded tRNA isoleucine</th></tr><tr><th colspan="2" class="infobox-header" style="background-color: #ddd">Identifiers</th></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3">Symbol</th><td class="infobox-data" style="background-color: #eee">MT-TI</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3">Alt. symbols</th><td class="infobox-data" style="background-color: #eee">MTTI</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/National_Center_for_Biotechnology_Information#Gene" title="National Center for Biotechnology Information">NCBI gene</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/gene?cmd=retrieve&amp;dopt=default&amp;list_uids=4565&amp;rn=1">4565</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/HUGO_Gene_Nomenclature_Committee" title="HUGO Gene Nomenclature Committee">HGNC</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:7488">7488</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/RefSeq" title="RefSeq">RefSeq</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/protein/NC_001807">NC_001807</a></td></tr><tr><th colspan="2" class="infobox-header" style="background-color: #ddd">Other data</th></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Locus_(genetics)" title="Locus (genetics)">Locus</a></th><td class="infobox-data" style="background-color: #eee"><a href="/wiki/Chromosome_MT_(human)" class="mw-redirect" title="Chromosome MT (human)">Chr. MT</a> <i><a rel="nofollow" class="external autonumber" href="https://omim.org/search/?index=geneMap&amp;search=MT">[1]</a></i></td></tr></tbody></table> <p><b>Mitochondrially encoded tRNA isoleucine</b> also known as <b>MT-TI</b> is a <a href="/wiki/Transfer_RNA" title="Transfer RNA">transfer RNA</a> which in humans is encoded by the <a href="/wiki/Mitochondrion" title="Mitochondrion">mitochondrial</a> <i>MT-TI</i> <a href="/wiki/Gene" title="Gene">gene</a>.<sup id="cite_ref-pmid7219534_1-0" class="reference"><a href="#cite_note-pmid7219534-1"><span class="cite-bracket">&#91;</span>1<span class="cite-bracket">&#93;</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Structure">Structure</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TI&amp;action=edit&amp;section=1" title="Edit section: Structure"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The <i>MT-TI</i> gene is located on the <a href="/wiki/Locus_(genetics)" title="Locus (genetics)">p arm</a> of the <a href="/wiki/Mitochondrial_DNA" title="Mitochondrial DNA">mitochondrial DNA</a> at position 12 and it spans 69 base pairs.<sup id="cite_ref-entrez_2-0" class="reference"><a href="#cite_note-entrez-2"><span class="cite-bracket">&#91;</span>2<span class="cite-bracket">&#93;</span></a></sup> The structure of a <a href="/wiki/TRNA" class="mw-redirect" title="TRNA">tRNA</a> molecule is a distinctive folded structure which contains three hairpin loops and resembles a three-leafed <a href="/wiki/Clover" title="Clover">clover</a>.<sup id="cite_ref-3" class="reference"><a href="#cite_note-3"><span class="cite-bracket">&#91;</span>3<span class="cite-bracket">&#93;</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Function">Function</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TI&amp;action=edit&amp;section=2" title="Edit section: Function"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>MT-TI is a small 69 nucleotide <a href="/wiki/RNA" title="RNA">RNA</a> (human mitochondrial map position 4263-4331) that transfers the amino acid <a href="/wiki/Isoleucine" title="Isoleucine">isoleucine</a> to a growing <a href="/wiki/Polypeptide" class="mw-redirect" title="Polypeptide">polypeptide</a> chain at the <a href="/wiki/Ribosomal" class="mw-redirect" title="Ribosomal">ribosome</a> site of <a href="/wiki/Protein" title="Protein">protein</a> synthesis during <a href="/wiki/Translation_(genetics)" class="mw-redirect" title="Translation (genetics)">translation</a>. </p> <div class="mw-heading mw-heading2"><h2 id="Clinical_significance">Clinical significance</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TI&amp;action=edit&amp;section=3" title="Edit section: Clinical significance"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Mutations in <i>MT-TI</i> can result in multiple mitochondrial deficiencies and associated disorders. </p> <div class="mw-heading mw-heading3"><h3 id="Myoclonic_epilepsy_with_ragged-red_fibers_(MERRF)"><span id="Myoclonic_epilepsy_with_ragged-red_fibers_.28MERRF.29"></span>Myoclonic epilepsy with ragged-red fibers (MERRF)</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TI&amp;action=edit&amp;section=4" title="Edit section: Myoclonic epilepsy with ragged-red fibers (MERRF)"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Mutations in the <i>MT-TI</i> gene have been associated with <a href="/wiki/Mitochondrial_myopathy" title="Mitochondrial myopathy">myoclonic epilepsy with ragged-red fibers (MERRF)</a>. Myoclonic epilepsy with ragged-red fibers (MERRF) is a disorder that affects many parts of the body, particularly the muscles and <a href="/wiki/Nervous_system" title="Nervous system">nervous system</a>. In most cases, the signs and symptoms of this disorder appear during childhood or <a href="/wiki/Adolescence" title="Adolescence">adolescence</a>. The features of MERRF vary widely among affected individuals, even among members of the same family. Common symptoms include, <a href="/wiki/Myoclonus" title="Myoclonus">myoclonus</a>, <a href="/wiki/Myopathy" title="Myopathy">myopathy</a>, <a href="/wiki/Spasticity" title="Spasticity">spasticity</a>, <a href="/wiki/Epilepsy" title="Epilepsy">epilepsy</a>, <a href="/wiki/Peripheral_neuropathy" title="Peripheral neuropathy">peripheral neuropathy</a>, <a href="/wiki/Dementia" title="Dementia">dementia</a>, <a href="/wiki/Ataxia" title="Ataxia">ataxia</a>, <a href="/wiki/Atrophy" title="Atrophy">atrophy</a>, and more.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">&#91;</span>4<span class="cite-bracket">&#93;</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Cardiomyopathy">Cardiomyopathy</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TI&amp;action=edit&amp;section=5" title="Edit section: Cardiomyopathy"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Mutations in the <i>MT-TI</i> gene may also cause <a href="/wiki/Cardiomyopathy" title="Cardiomyopathy">cardiomyopathy</a>, a disorder of the <a href="/wiki/Heart" title="Heart">heart</a> characterized by the thickening of the heart, usually in the <a href="/wiki/Interventricular_septum" title="Interventricular septum">interventricular septum</a>, which results in a weakened heart muscle that is unable to pump blood effectively. It is unclear why such mutations result in the symptoms of isolated cardiomyopathy.<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">&#91;</span>5<span class="cite-bracket">&#93;</span></a></sup> Mutations of 4300A&gt;G, 4295A&gt;G, 4269A&gt;G, and 4317A&gt;G in the <i>MT-TI</i> gene have been found in patients with cardiomyopathy in varying severities and onset.<sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">&#91;</span>6<span class="cite-bracket">&#93;</span></a></sup><sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">&#91;</span>7<span class="cite-bracket">&#93;</span></a></sup><sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">&#91;</span>8<span class="cite-bracket">&#93;</span></a></sup><sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">&#91;</span>9<span class="cite-bracket">&#93;</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Complex_IV_Deficiency">Complex IV Deficiency</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TI&amp;action=edit&amp;section=6" title="Edit section: Complex IV Deficiency"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><i>MT-TI</i> mutations have been associated with complex IV deficiency of the <a href="/wiki/Mitochondrial_respiratory_chain" class="mw-redirect" title="Mitochondrial respiratory chain">mitochondrial respiratory chain</a>, also known as the <a href="/wiki/Cytochrome_c_oxidase" title="Cytochrome c oxidase">cytochrome c oxidase</a> deficiency. Cytochrome c oxidase deficiency is a rare genetic condition that can affect multiple parts of the body, including <a href="/wiki/Skeletal_muscles" class="mw-redirect" title="Skeletal muscles">skeletal muscles</a>, the <a href="/wiki/Heart" title="Heart">heart</a>, the <a href="/wiki/Brain" title="Brain">brain</a>, or the <a href="/wiki/Liver" title="Liver">liver</a>. Common clinical manifestations include <a href="/wiki/Myopathy" title="Myopathy">myopathy</a>, <a href="/wiki/Hypotonia" title="Hypotonia">hypotonia</a>, and <a href="/wiki/Encephalomyopathy" class="mw-redirect" title="Encephalomyopathy">encephalomyopathy</a>, <a href="/wiki/Lactic_acidosis" title="Lactic acidosis">lactic acidosis</a>, and <a href="/wiki/Hypertrophic_cardiomyopathy" title="Hypertrophic cardiomyopathy">hypertrophic cardiomyopathy</a>.<sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">&#91;</span>10<span class="cite-bracket">&#93;</span></a></sup> A patient with a 4269A&gt;G mutation in <i>MT-TI</i> was found with the deficiency.<sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">&#91;</span>11<span class="cite-bracket">&#93;</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=MT-TI&amp;action=edit&amp;section=7" 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 reflist-columns references-column-width" style="column-width: 32em;"> <ol class="references"> <li id="cite_note-pmid7219534-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-pmid7219534_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 id="CITEREFAndersonBankierBarrellde_Bruijn1981" class="citation journal cs1">Anderson S, Bankier AT, Barrell BG, de Bruijn MH, Coulson AR, Drouin J, et&#160;al. 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title="ctx_ver=Z39.88-2004&amp;rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&amp;rft.genre=article&amp;rft.jtitle=Journal+of+the+American+College+of+Cardiology&amp;rft.atitle=A+homoplasmic+mitochondrial+transfer+ribonucleic+acid+mutation+as+a+cause+of+maternally+inherited+hypertrophic+cardiomyopathy&amp;rft.volume=41&amp;rft.issue=10&amp;rft.pages=%3Cspan+class%3D%22nowrap%22%3E1786-%3C%2Fspan%3E96&amp;rft.date=2003-05&amp;rft_id=info%3Adoi%2F10.1016%2FS0735-1097%2803%2900300-0&amp;rft_id=info%3Apmid%2F12767666&amp;rft.aulast=Taylor&amp;rft.aufirst=RW&amp;rft.au=Giordano%2C+C&amp;rft.au=Davidson%2C+MM&amp;rft.au=d%27Amati%2C+G&amp;rft.au=Bain%2C+H&amp;rft.au=Hayes%2C+CM&amp;rft.au=Leonard%2C+H&amp;rft.au=Barron%2C+MJ&amp;rft.au=Casali%2C+C&amp;rft.au=Santorelli%2C+FM&amp;rft.au=Hirano%2C+M&amp;rft.au=Lightowlers%2C+RN&amp;rft.au=DiMauro%2C+S&amp;rft.au=Turnbull%2C+DM&amp;rft_id=https%3A%2F%2Fdoi.org%2F10.1016%252FS0735-1097%252803%252900300-0&amp;rfr_id=info%3Asid%2Fen.wikipedia.org%3AMT-TI" class="Z3988"></span></span> </li> <li id="cite_note-10"><span class="mw-cite-backlink"><b><a href="#cite_ref-10">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite id="CITEREFReference" class="citation web cs1">Reference, Genetics Home. <a rel="nofollow" class="external text" href="https://ghr.nlm.nih.gov/condition/cytochrome-c-oxidase-deficiency">"Cytochrome c oxidase deficiency"</a>. <i>Genetics Home Reference</i>.</cite><span title="ctx_ver=Z39.88-2004&amp;rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&amp;rft.genre=unknown&amp;rft.jtitle=Genetics+Home+Reference&amp;rft.atitle=Cytochrome+c+oxidase+deficiency&amp;rft.aulast=Reference&amp;rft.aufirst=Genetics+Home&amp;rft_id=https%3A%2F%2Fghr.nlm.nih.gov%2Fcondition%2Fcytochrome-c-oxidase-deficiency&amp;rfr_id=info%3Asid%2Fen.wikipedia.org%3AMT-TI" class="Z3988"></span><span class="noviewer" typeof="mw:File"><span><img alt="Public Domain" src="//upload.wikimedia.org/wikipedia/en/thumb/6/62/PD-icon.svg/12px-PD-icon.svg.png" decoding="async" width="12" height="12" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/6/62/PD-icon.svg/18px-PD-icon.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/6/62/PD-icon.svg/24px-PD-icon.svg.png 2x" data-file-width="196" data-file-height="196" /></span></span> This article incorporates text from this source, which is in the <a href="/wiki/Public_domain" title="Public domain">public domain</a>.</span> </li> <li id="cite_note-11"><span class="mw-cite-backlink"><b><a href="#cite_ref-11">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite id="CITEREFKaidoFujimuraTaniikeYoshikawa1995" class="citation journal cs1">Kaido M, Fujimura H, Taniike M, Yoshikawa H, Toyooka K, Yorifuji S, Inui K, Okada S, Sparaco M, Yanagihara T (August 1995). <a rel="nofollow" class="external text" href="https://doi.org/10.1016%2F0022-510X%2895%2900111-E">"Focal cytochrome c oxidase deficiency in the brain and dorsal root ganglia in a case with mitochondrial encephalomyopathy (tRNA(Ile) 4269 mutation): histochemical, immunohistochemical, and ultrastructural study"</a>. <i>Journal of the Neurological Sciences</i>. <b>131</b> (2): <span class="nowrap">170–</span>6. <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/10.1016%2F0022-510X%2895%2900111-E">10.1016/0022-510X(95)00111-E</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a>&#160;<a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/7595643">7595643</a>. <a href="/wiki/S2CID_(identifier)" class="mw-redirect" title="S2CID (identifier)">S2CID</a>&#160;<a rel="nofollow" class="external text" href="https://api.semanticscholar.org/CorpusID:41493869">41493869</a>.</cite><span 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class="Z3988"></span></span> </li> </ol></div> <div class="mw-heading mw-heading2"><h2 id="Further_reading">Further reading</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TI&amp;action=edit&amp;section=8" title="Edit section: Further reading"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite id="CITEREFFinsterer2003" class="citation journal cs1">Finsterer, J (January 2003). "Mitochondriopathy mimicking amyotrophic lateral sclerosis". <i>The Neurologist</i>. <b>9</b> (1): <span class="nowrap">45–</span>8. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1097%2F01.nrl.0000038589.58012.a8">10.1097/01.nrl.0000038589.58012.a8</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a>&#160;<a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/12801431">12801431</a>. <a href="/wiki/S2CID_(identifier)" class="mw-redirect" title="S2CID (identifier)">S2CID</a>&#160;<a rel="nofollow" class="external text" href="https://api.semanticscholar.org/CorpusID:43440359">43440359</a>.</cite><span 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href="/wiki/Kynureninase" title="Kynureninase">Kynureninase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Monoamine_neurotransmitter" title="Monoamine neurotransmitter">monoamine neurotransmitter</a><br /> metabolism</i></span></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/Monoamine_oxidase" title="Monoamine oxidase">Monoamine oxidase</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mitochondrial_intermembrane_space" class="mw-redirect" title="Mitochondrial intermembrane space">Intermembrane space</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/Adenylate_kinase" title="Adenylate kinase">Adenylate kinase</a></li> <li><a href="/wiki/Creatine_kinase" title="Creatine kinase">Creatine kinase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Inner_mitochondrial_membrane" title="Inner mitochondrial membrane">Inner membrane</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 scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Oxidative_phosphorylation" title="Oxidative phosphorylation">oxidative phosphorylation</a></i></span></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/Coenzyme_Q_%E2%80%93_cytochrome_c_reductase" title="Coenzyme Q – cytochrome c reductase">Coenzyme Q – cytochrome c reductase</a></li> <li><a href="/wiki/Cytochrome_c" title="Cytochrome c">Cytochrome c</a></li> <li><a href="/wiki/NADH_dehydrogenase" title="NADH dehydrogenase">NADH dehydrogenase</a></li> <li><a href="/wiki/Succinate_dehydrogenase" title="Succinate dehydrogenase">Succinate dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Pyrimidine_metabolism" title="Pyrimidine metabolism">pyrimidine metabolism</a></i></span></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/Dihydroorotate_dehydrogenase" title="Dihydroorotate dehydrogenase">Dihydroorotate dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Mitochondrial_shuttle" title="Mitochondrial shuttle">mitochondrial shuttle</a></i></span></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/Malate-aspartate_shuttle" class="mw-redirect" title="Malate-aspartate shuttle">Malate-aspartate shuttle</a></li> <li><a href="/wiki/Glycerol_phosphate_shuttle" title="Glycerol phosphate shuttle">Glycerol phosphate shuttle</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Steroidogenesis" class="mw-redirect" title="Steroidogenesis">steroidogenesis</a></i></span></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/Cholesterol_side-chain_cleavage_enzyme" title="Cholesterol side-chain cleavage enzyme">Cholesterol side-chain cleavage enzyme</a></li> <li><a href="/wiki/Steroid_11-beta-hydroxylase" class="mw-redirect" title="Steroid 11-beta-hydroxylase">Steroid 11-beta-hydroxylase</a></li> <li><a href="/wiki/Aldosterone_synthase" title="Aldosterone synthase">Aldosterone synthase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>other</i></span></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/Glutamate_aspartate_transporter" class="mw-redirect" title="Glutamate aspartate transporter">Glutamate aspartate transporter</a></li> <li><a href="/wiki/Glycerol-3-phosphate_dehydrogenase" title="Glycerol-3-phosphate dehydrogenase">Glycerol-3-phosphate dehydrogenase</a></li> <li><a href="/wiki/ATP_synthase" title="ATP synthase">ATP synthase</a></li> <li><a href="/wiki/Carnitine_palmitoyltransferase_II" title="Carnitine palmitoyltransferase II">Carnitine palmitoyltransferase II</a></li> <li><a href="/wiki/Uncoupling_protein" title="Uncoupling protein">Uncoupling protein</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mitochondrial_matrix" title="Mitochondrial matrix">Matrix</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 scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Citric_acid_cycle" title="Citric acid cycle">citric acid cycle</a></i></span></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/Citrate_synthase" title="Citrate synthase">Citrate synthase</a></li> <li><a href="/wiki/Aconitase" title="Aconitase">Aconitase</a></li> <li><a href="/wiki/Isocitrate_dehydrogenase" title="Isocitrate dehydrogenase">Isocitrate dehydrogenase</a></li> <li><a href="/wiki/Oxoglutarate_dehydrogenase_complex" title="Oxoglutarate dehydrogenase complex">Oxoglutarate dehydrogenase complex</a></li> <li><a href="/wiki/Succinyl_coenzyme_A_synthetase" title="Succinyl coenzyme A synthetase">Succinyl coenzyme A synthetase</a></li> <li><a href="/wiki/Fumarase" title="Fumarase">Fumarase</a></li> <li><a href="/wiki/Malate_dehydrogenase" title="Malate dehydrogenase">Malate dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Anaplerotic_reactions" title="Anaplerotic reactions">anaplerotic reactions</a></i></span></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/Aspartate_transaminase" title="Aspartate transaminase">Aspartate transaminase</a></li> <li><a href="/wiki/Glutamate_dehydrogenase" title="Glutamate dehydrogenase">Glutamate dehydrogenase</a></li> <li><a href="/wiki/Pyruvate_dehydrogenase_complex" title="Pyruvate dehydrogenase complex">Pyruvate dehydrogenase complex</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Urea_cycle" title="Urea cycle">urea cycle</a></i></span></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/Carbamoyl_phosphate_synthetase_I" title="Carbamoyl phosphate synthetase I">Carbamoyl phosphate synthetase I</a></li> <li><a href="/wiki/Ornithine_transcarbamylase" title="Ornithine transcarbamylase">Ornithine transcarbamylase</a></li> <li><a href="/wiki/N-Acetylglutamate_synthase" title="N-Acetylglutamate synthase">N-Acetylglutamate synthase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>alcohol metabolism</i></span></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/ALDH2" title="ALDH2">ALDH2</a></li></ul> </div></td></tr><tr><td colspan="2" class="navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/PMPCB" title="PMPCB">PMPCB</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other/to be sorted</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><td colspan="2" class="navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Frataxin" title="Frataxin">Frataxin</a></li> <li><a href="/wiki/Mitochondrial_membrane_transport_protein" title="Mitochondrial membrane transport protein">Mitochondrial membrane transport protein</a> <ul><li><a href="/wiki/Mitochondrial_permeability_transition_pore" title="Mitochondrial permeability transition pore">Mitochondrial permeability transition pore</a></li> <li><a href="/wiki/Mitochondrial_carrier" title="Mitochondrial carrier">Mitochondrial carrier</a></li></ul></li> <li><a href="/wiki/Translocator_protein" title="Translocator protein">Translocator protein</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mitochondrial_DNA" title="Mitochondrial DNA">Mitochondrial DNA</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 scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/NADH_dehydrogenase" title="NADH dehydrogenase">Complex I</a></span></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/MT-ND1" title="MT-ND1">MT-ND1</a></li> <li><a href="/wiki/MT-ND2" title="MT-ND2">MT-ND2</a></li> <li><a href="/wiki/MT-ND3" title="MT-ND3">MT-ND3</a></li> <li><a href="/wiki/MT-ND4" title="MT-ND4">MT-ND4</a></li> <li><a href="/wiki/MT-ND4L" title="MT-ND4L">MT-ND4L</a></li> <li><a href="/wiki/MT-ND5" title="MT-ND5">MT-ND5</a></li> <li><a href="/wiki/MT-ND6" title="MT-ND6">MT-ND6</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/Coenzyme_Q_%E2%80%93_cytochrome_c_reductase" title="Coenzyme Q – cytochrome c reductase">Complex III</a></span></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/MT-CYB" title="MT-CYB">MT-CYB</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/Cytochrome_c_oxidase" title="Cytochrome c oxidase">Complex IV</a></span></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/Cytochrome_c_oxidase_subunit_I" title="Cytochrome c oxidase subunit I">MT-CO1</a></li> <li><a href="/wiki/Cytochrome_c_oxidase_subunit_II" class="mw-redirect" title="Cytochrome c oxidase subunit II">MT-CO2</a></li> <li><a href="/wiki/Cytochrome_c_oxidase_subunit_III" title="Cytochrome c oxidase subunit III">MT-CO3</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/ATP_synthase" title="ATP synthase">ATP synthase</a></span></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/MT-ATP6" title="MT-ATP6">MT-ATP6</a></li> <li><a href="/wiki/MT-ATP8" title="MT-ATP8">MT-ATP8</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/Transfer_RNA" title="Transfer RNA">tRNA</a></span></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/MT-TA" title="MT-TA">MT-TA</a></li> <li><a href="/wiki/MT-TC" title="MT-TC">MT-TC</a></li> <li><a href="/wiki/MT-TD" title="MT-TD">MT-TD</a></li> <li><a href="/wiki/MT-TE" title="MT-TE">MT-TE</a></li> <li><a href="/wiki/MT-TF" title="MT-TF">MT-TF</a></li> <li><a href="/wiki/MT-TG" title="MT-TG">MT-TG</a></li> <li><a href="/wiki/MT-TH" title="MT-TH">MT-TH</a></li> <li><a class="mw-selflink selflink">MT-TI</a></li> <li><a href="/wiki/MT-TK" title="MT-TK">MT-TK</a></li> <li><a href="/wiki/MT-TL1" title="MT-TL1">MT-TL1</a></li> <li><a href="/wiki/MT-TL2" title="MT-TL2">MT-TL2</a></li> <li><a href="/wiki/MT-TM" title="MT-TM">MT-TM</a></li> <li><a href="/wiki/MT-TN" title="MT-TN">MT-TN</a></li> <li><a href="/wiki/MT-TP" title="MT-TP">MT-TP</a></li> <li><a href="/wiki/MT-TQ" title="MT-TQ">MT-TQ</a></li> <li><a href="/wiki/MT-TR" title="MT-TR">MT-TR</a></li> <li><a href="/wiki/MT-TS1" title="MT-TS1">MT-TS1</a></li> <li><a href="/wiki/MT-TS2" title="MT-TS2">MT-TS2</a></li> <li><a href="/wiki/MT-TT" title="MT-TT">MT-TT</a></li> <li><a href="/wiki/MT-TV_(mitochondrial)" title="MT-TV (mitochondrial)">MT-TV</a></li> <li><a href="/wiki/MT-TW" title="MT-TW">MT-TW</a></li> <li><a href="/wiki/MT-TY" title="MT-TY">MT-TY</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><td class="navbox-abovebelow" colspan="2" style="padding:0;"><div><i>see also <a href="/wiki/Template:Mitochondrial_diseases" title="Template:Mitochondrial diseases">mitochondrial diseases</a></i></div></td></tr></tbody></table></div> <p><i>This article incorporates text from the <a href="/wiki/United_States_National_Library_of_Medicine" title="United States National Library of Medicine">United States National Library of Medicine</a>, which is in the <a href="/wiki/Public_domain" title="Public domain">public domain</a>.</i> </p> <!-- NewPP limit report Parsed by mw‐api‐ext.eqiad.main‐6ff9949d66‐hm5wz Cached time: 20250306023134 Cache expiry: 2592000 Reduced expiry: false Complications: [vary‐revision‐sha1, show‐toc] CPU time usage: 0.311 seconds Real time usage: 0.458 seconds Preprocessor visited node count: 1526/1000000 Post‐expand include size: 80055/2097152 bytes Template argument size: 1138/2097152 bytes Highest expansion depth: 9/100 Expensive parser function count: 1/500 Unstrip recursion depth: 1/20 Unstrip post‐expand size: 56245/5000000 bytes Lua time usage: 0.195/10.000 seconds Lua memory usage: 5145970/52428800 bytes Number of Wikibase entities loaded: 1/400 --> <!-- Transclusion expansion time report (%,ms,calls,template) 100.00% 388.236 1 -total 35.51% 137.881 1 Template:Reflist 26.53% 103.007 7 Template:Cite_journal 23.84% 92.562 6 Template:Navbox 23.06% 89.512 1 Template:Mitochondrial_enzymes 20.23% 78.546 1 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