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data-event-name="pinnable-header.vector-toc.pin">move to sidebar</button> <button class="vector-pinnable-header-toggle-button vector-pinnable-header-unpin-button" data-event-name="pinnable-header.vector-toc.unpin">hide</button> </div> <ul class="vector-toc-contents" id="mw-panel-toc-list"> <li id="toc-mw-content-text" class="vector-toc-list-item vector-toc-level-1"> <a href="#" class="vector-toc-link"> <div class="vector-toc-text">(Top)</div> </a> </li> <li id="toc-Structure" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Structure"> <div class="vector-toc-text"> <span class="vector-toc-numb">1</span> <span>Structure</span> </div> </a> <ul id="toc-Structure-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Function" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Function"> <div class="vector-toc-text"> <span class="vector-toc-numb">2</span> <span>Function</span> </div> </a> <ul id="toc-Function-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Clinical_significance" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Clinical_significance"> <div class="vector-toc-text"> <span class="vector-toc-numb">3</span> <span>Clinical significance</span> </div> </a> <button aria-controls="toc-Clinical_significance-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 Clinical significance subsection</span> </button> <ul id="toc-Clinical_significance-sublist" class="vector-toc-list"> <li id="toc-Mitochondrial_encephalomyopathy,_lactic_acidosis,_and_stroke-like_episodes_(MELAS)" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Mitochondrial_encephalomyopathy,_lactic_acidosis,_and_stroke-like_episodes_(MELAS)"> <div class="vector-toc-text"> <span class="vector-toc-numb">3.1</span> <span>Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)</span> </div> </a> <ul id="toc-Mitochondrial_encephalomyopathy,_lactic_acidosis,_and_stroke-like_episodes_(MELAS)-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-MERRF/MELAS_overlap_syndrome" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#MERRF/MELAS_overlap_syndrome"> <div class="vector-toc-text"> <span class="vector-toc-numb">3.2</span> <span>MERRF/MELAS overlap syndrome</span> </div> </a> <ul id="toc-MERRF/MELAS_overlap_syndrome-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Cardiomyopathy" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Cardiomyopathy"> <div class="vector-toc-text"> <span class="vector-toc-numb">3.3</span> <span>Cardiomyopathy</span> </div> </a> <ul id="toc-Cardiomyopathy-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Deafness,_Nonsyndromic_Sensorineural,_Mitochondrial" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Deafness,_Nonsyndromic_Sensorineural,_Mitochondrial"> <div class="vector-toc-text"> <span class="vector-toc-numb">3.4</span> <span>Deafness, Nonsyndromic Sensorineural, Mitochondrial</span> </div> </a> <ul id="toc-Deafness,_Nonsyndromic_Sensorineural,_Mitochondrial-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">4</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 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</div> </div> <div id="bodyContent" class="vector-body" aria-labelledby="firstHeading" data-mw-ve-target-container> <div class="vector-body-before-content"> <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 in the species Homo sapiens</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 histidine</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-TH</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3">Alt. symbols</th><td class="infobox-data" style="background-color: #eee">MTTH</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&dopt=default&list_uids=4564&rn=1">4564</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:7487">7487</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://omim.org/entry/590040">590040</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&search=MT">[1]</a></i></td></tr></tbody></table> <p><b>Mitochondrially encoded tRNA histidine</b>, also known as <b>MT-TH</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-TH</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">[</span>1<span class="cite-bracket">]</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-TH&action=edit&section=1" title="Edit section: Structure"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The <i>MT-TH</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">[</span>2<span class="cite-bracket">]</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">[</span>3<span class="cite-bracket">]</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-TH&action=edit&section=2" title="Edit section: Function"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>MT-TH is a small 69 nucleotide <a href="/wiki/Transfer_RNA" title="Transfer RNA">transfer RNA</a> (human mitochondrial map position 12138–12206) that transfers the amino acid <a href="/wiki/Histidine" title="Histidine">histidine</a> to a growing <a href="/wiki/Polypeptide" class="mw-redirect" title="Polypeptide">polypeptide</a> at the <a href="/wiki/Ribosome" title="Ribosome">ribosomal</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>.<sup id="cite_ref-BlakelyYarham2013_4-0" class="reference"><a href="#cite_note-BlakelyYarham2013-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </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-TH&action=edit&section=3" title="Edit section: Clinical significance"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Mutations in <i>MT-TH</i> can result in multiple mitochondrial deficiencies and associated disorders. MT-TH is associated with <a href="/wiki/Mitochondrial_encephalomyopathy,_lactic_acidosis,_and_stroke-like_episodes" class="mw-redirect" title="Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes">mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes</a> (MELAS),<sup id="cite_ref-pmid14967777_5-0" class="reference"><a href="#cite_note-pmid14967777-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-pmid15111688_6-0" class="reference"><a href="#cite_note-pmid15111688-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Cardiomyopathy" title="Cardiomyopathy">cardiomyopathy</a>, and the MELAS/MERRF overlap syndrome.<sup id="cite_ref-GHR_7-0" class="reference"><a href="#cite_note-GHR-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Mitochondrial_encephalomyopathy,_lactic_acidosis,_and_stroke-like_episodes_(MELAS)"><span id="Mitochondrial_encephalomyopathy.2C_lactic_acidosis.2C_and_stroke-like_episodes_.28MELAS.29"></span>Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TH&action=edit&section=4" title="Edit section: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>A small number of people with symptoms of <a href="/wiki/Mitochondrial_encephalomyopathy,_lactic_acidosis,_and_stroke-like_episodes" class="mw-redirect" title="Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes">mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)</a> have been found to have mutations in the <i>MT-TH</i> gene. MELAS is a rare mitochondrial disorder known to affect many parts of the body, especially the <a href="/wiki/Nervous_system" title="Nervous system">nervous system</a> and the <a href="/wiki/Brain" title="Brain">brain</a>. Symptoms of MELAS include recurrent severe <a href="/wiki/Headaches" class="mw-redirect" title="Headaches">headaches</a>, muscle weakness (<a href="/wiki/Myopathy" title="Myopathy">myopathy</a>), <a href="/wiki/Hearing_loss" title="Hearing loss">hearing loss</a>, <a href="/wiki/Stroke" title="Stroke">stroke</a>-like episodes with a loss of <a href="/wiki/Consciousness" title="Consciousness">consciousness</a>, <a href="/wiki/Seizures" class="mw-redirect" title="Seizures">seizures</a>, and other problems affecting the <a href="/wiki/Nervous_system" title="Nervous system">nervous system</a>.<sup id="cite_ref-GHR_7-1" class="reference"><a href="#cite_note-GHR-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="MERRF/MELAS_overlap_syndrome"><span id="MERRF.2FMELAS_overlap_syndrome"></span>MERRF/MELAS overlap syndrome</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TH&action=edit&section=5" title="Edit section: MERRF/MELAS overlap syndrome"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>MELAS syndrome may also be accompanied by another mitochondrial disorder called <a href="/wiki/MERRF_syndrome" title="MERRF syndrome">myoclonic epilepsy with ragged-red fibers</a>, also known as <a href="/wiki/MERRF_syndrome" title="MERRF syndrome">MERRF syndrome</a>.<sup id="cite_ref-pmid14967777_5-1" class="reference"><a href="#cite_note-pmid14967777-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> In addition to symptoms of MELAS syndrome, additional signs and symptoms may include muscle twitches (<a href="/wiki/Myoclonus" title="Myoclonus">myoclonus</a>), difficulty coordinating movement (<a href="/wiki/Ataxia" title="Ataxia">ataxia</a>), and abnormal muscle cells known as <a href="/wiki/Ragged-red_fibers" class="mw-redirect" title="Ragged-red fibers">ragged-red fibers</a>. The combination of MERRF and MELAS is called the MERRF/MELAS overlap syndrome, which is caused by mutations in the <i>MT-TH</i> gene. It has not been determined how such mutations alter the energy production function of the <a href="/wiki/Mitochondria" class="mw-redirect" title="Mitochondria">mitochondria</a> and result in symptoms of such syndromes.<sup id="cite_ref-GHR_7-2" class="reference"><a href="#cite_note-GHR-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> A specific mutation of 12147G>A in the <i>MT-TH</i> gene has been found to result in the MERRF/MELAS overlap syndrome. A patient with the mutation exhibited symptoms of migrainous <a href="/wiki/Headache" title="Headache">headache</a> and vomiting, left <a href="/wiki/Hemiparesis" title="Hemiparesis">hemiparesis</a>, lateral homonymous <a href="/wiki/Hemianopia" class="mw-redirect" title="Hemianopia">hemianopia</a>, and others consistent with the MERRF/MELAS overlap syndrome. The patient exhibited symptoms of MELAS first, then progressed into the overlap syndrome.<sup id="cite_ref-pmid14967777_5-2" class="reference"><a href="#cite_note-pmid14967777-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</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-TH&action=edit&section=6" title="Edit section: Cardiomyopathy"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Mutations in the <i>MT-TH</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. Patients with mutations in the <i>MT-TH</i> gene have been found to exhibit symptoms of cardiomyopathy without other common signs of mitochondrial disease such as neurological abnormalities. It is unclear why such mutations result in the symptoms of isolated cardiomyopathy.<sup id="cite_ref-GHR_7-3" class="reference"><a href="#cite_note-GHR-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> A specific mutation of 12192G>A in the <i>MT-TH</i> gene has been found in multiple patients with the disorder. patients exhibited symptoms of <a href="/wiki/Cardiomyopathy" title="Cardiomyopathy">cardiomyopathy</a> in different forms.<sup id="cite_ref-pmid11038324_8-0" class="reference"><a href="#cite_note-pmid11038324-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Deafness,_Nonsyndromic_Sensorineural,_Mitochondrial"><span id="Deafness.2C_Nonsyndromic_Sensorineural.2C_Mitochondrial"></span>Deafness, Nonsyndromic Sensorineural, Mitochondrial</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=MT-TH&action=edit&section=7" title="Edit section: Deafness, Nonsyndromic Sensorineural, Mitochondrial"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Deafness has also been associated with mutations in the <i>MT-TH</i> gene. Heteroplasmic 12201T>C transitions in <i>MT-TH</i> have been found in a family exhibiting symptoms of nonsyndromic <a href="/wiki/Sensorineural_deafness" class="mw-redirect" title="Sensorineural deafness">sensorineural deafness</a>, varying in time of onset and severity.<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> </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-TH&action=edit&section=8" 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"><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 al. 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title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Neurology&rft.atitle=Catastrophic+presentation+of+mitochondrial+disease+due+to+a+mutation+in+the+tRNA%28His%29+gene&rft.volume=62&rft.issue=8&rft.pages=1420-3&rft.date=2004-04&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A46681236%23id-name%3DS2CID&rft_id=info%3Apmid%2F15111688&rft_id=info%3Adoi%2F10.1212%2F01.wnl.0000120667.77372.46&rft.aulast=Taylor&rft.aufirst=RW&rft.au=Schaefer%2C+AM&rft.au=McDonnell%2C+MT&rft.au=Petty%2C+RK&rft.au=Thomas%2C+AM&rft.au=Blakely%2C+EL&rft.au=Hayes%2C+CM&rft.au=McFarland%2C+R&rft.au=Turnbull%2C+DM&rft_id=http%3A%2F%2Fwww.neurology.org%2Fcgi%2Fcontent%2Fabstract%2F62%2F8%2F1420&rfr_id=info%3Asid%2Fen.wikipedia.org%3AMT-TH" class="Z3988"></span></span> </li> <li id="cite_note-GHR-7"><span class="mw-cite-backlink">^ <a href="#cite_ref-GHR_7-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-GHR_7-1"><sup><i><b>b</b></i></sup></a> <a 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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-pmid11038324-8"><span class="mw-cite-backlink"><b><a href="#cite_ref-pmid11038324_8-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFShinTanakaSuzukiHemmi2000" class="citation journal cs1">Shin WS, Tanaka M, Suzuki J, Hemmi C, Toyo-oka T (December 2000). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1287941">"A novel homoplasmic mutation in mtDNA with a single evolutionary origin as a risk factor for cardiomyopathy"</a>. <i>American Journal of Human Genetics</i>. <b>67</b> (6): 1617–20. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1086%2F316896">10.1086/316896</a>. <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/PMC1287941">1287941</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/11038324">11038324</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=American+Journal+of+Human+Genetics&rft.atitle=A+novel+homoplasmic+mutation+in+mtDNA+with+a+single+evolutionary+origin+as+a+risk+factor+for+cardiomyopathy&rft.volume=67&rft.issue=6&rft.pages=1617-20&rft.date=2000-12&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1287941%23id-name%3DPMC&rft_id=info%3Apmid%2F11038324&rft_id=info%3Adoi%2F10.1086%2F316896&rft.aulast=Shin&rft.aufirst=WS&rft.au=Tanaka%2C+M&rft.au=Suzuki%2C+J&rft.au=Hemmi%2C+C&rft.au=Toyo-oka%2C+T&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1287941&rfr_id=info%3Asid%2Fen.wikipedia.org%3AMT-TH" class="Z3988"></span></span> </li> <li id="cite_note-9"><span class="mw-cite-backlink"><b><a href="#cite_ref-9">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFYanWangWangSun2011" class="citation journal cs1">Yan X, Wang X, Wang Z, Sun S, Chen G, He Y, et al. (October 2011). "Maternally transmitted late-onset non-syndromic deafness is associated with the novel heteroplasmic T12201C mutation in the mitochondrial tRNAHis gene". <i>Journal of Medical Genetics</i>. <b>48</b> (10): 682–90. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1136%2Fjmedgenet-2011-100219">10.1136/jmedgenet-2011-100219</a>. <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/21931169">21931169</a>. <a href="/wiki/S2CID_(identifier)" class="mw-redirect" title="S2CID (identifier)">S2CID</a> <a rel="nofollow" class="external text" href="https://api.semanticscholar.org/CorpusID:13128611">13128611</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Journal+of+Medical+Genetics&rft.atitle=Maternally+transmitted+late-onset+non-syndromic+deafness+is+associated+with+the+novel+heteroplasmic+T12201C+mutation+in+the+mitochondrial+tRNAHis+gene&rft.volume=48&rft.issue=10&rft.pages=682-90&rft.date=2011-10&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A13128611%23id-name%3DS2CID&rft_id=info%3Apmid%2F21931169&rft_id=info%3Adoi%2F10.1136%2Fjmedgenet-2011-100219&rft.aulast=Yan&rft.aufirst=X&rft.au=Wang%2C+X&rft.au=Wang%2C+Z&rft.au=Sun%2C+S&rft.au=Chen%2C+G&rft.au=He%2C+Y&rft.au=Mo%2C+JQ&rft.au=Li%2C+R&rft.au=Jiang%2C+P&rft.au=Lin%2C+Q&rft.au=Sun%2C+M&rft.au=Li%2C+W&rft.au=Bai%2C+Y&rft.au=Zhang%2C+J&rft.au=Zhu%2C+Y&rft.au=Lu%2C+J&rft.au=Yan%2C+Q&rft.au=Li%2C+H&rft.au=Guan%2C+MX&rfr_id=info%3Asid%2Fen.wikipedia.org%3AMT-TH" class="Z3988"></span></span> </li> </ol></div></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-TH&action=edit&section=9" title="Edit section: Further reading"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239549316">.mw-parser-output .refbegin{margin-bottom:0.5em}.mw-parser-output .refbegin-hanging-indents>ul{margin-left:0}.mw-parser-output .refbegin-hanging-indents>ul>li{margin-left:0;padding-left:3.2em;text-indent:-3.2em}.mw-parser-output .refbegin-hanging-indents ul,.mw-parser-output .refbegin-hanging-indents ul li{list-style:none}@media(max-width:720px){.mw-parser-output .refbegin-hanging-indents>ul>li{padding-left:1.6em;text-indent:-1.6em}}.mw-parser-output .refbegin-columns{margin-top:0.3em}.mw-parser-output .refbegin-columns ul{margin-top:0}.mw-parser-output .refbegin-columns li{page-break-inside:avoid;break-inside:avoid-column}@media screen{.mw-parser-output .refbegin{font-size:90%}}</style><div class="refbegin" style=""> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFMeloneTessaPetriniLus2004" class="citation journal cs1">Melone MA, Tessa A, Petrini S, Lus G, Sampaolo S, di Fede G, Santorelli FM, Cotrufo R (February 2004). <a rel="nofollow" class="external text" href="https://doi.org/10.1001%2Farchneur.61.2.269">"Revelation of a new mitochondrial DNA mutation (G12147A) in a MELAS/MERFF phenotype"</a>. <i>Archives of Neurology</i>. <b>61</b> (2): 269–72. <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/10.1001%2Farchneur.61.2.269">10.1001/archneur.61.2.269</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/14967777">14967777</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Archives+of+Neurology&rft.atitle=Revelation+of+a+new+mitochondrial+DNA+mutation+%28G12147A%29+in+a+MELAS%2FMERFF+phenotype&rft.volume=61&rft.issue=2&rft.pages=269-72&rft.date=2004-02&rft_id=info%3Adoi%2F10.1001%2Farchneur.61.2.269&rft_id=info%3Apmid%2F14967777&rft.aulast=Melone&rft.aufirst=MA&rft.au=Tessa%2C+A&rft.au=Petrini%2C+S&rft.au=Lus%2C+G&rft.au=Sampaolo%2C+S&rft.au=di+Fede%2C+G&rft.au=Santorelli%2C+FM&rft.au=Cotrufo%2C+R&rft_id=https%3A%2F%2Fdoi.org%2F10.1001%252Farchneur.61.2.269&rfr_id=info%3Asid%2Fen.wikipedia.org%3AMT-TH" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFCalvarusoWillemsenRodenburgvan_den_Brand2011" class="citation journal cs1">Calvaruso MA, Willemsen MA, Rodenburg RJ, van den Brand M, Smeitink JA, Nijtmans L (September 2011). "New mitochondrial tRNA HIS mutation in a family with lactic acidosis and stroke-like episodes (MELAS)". <i>Mitochondrion</i>. <b>11</b> (5): 778–82. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.mito.2011.06.004">10.1016/j.mito.2011.06.004</a>. <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/21704194">21704194</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 class="mw-selflink selflink">MT-TH</a></li> <li><a href="/wiki/MT-TI" title="MT-TI">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 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