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N-Acetylglutamate synthase - Wikipedia

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class="interlanguage-link-target"><span>فارسی</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/N-ac%C3%A9tylglutamate_synthase" title="N-acétylglutamate synthase – French" lang="fr" hreflang="fr" data-title="N-acétylglutamate synthase" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-it mw-list-item"><a href="https://it.wikipedia.org/wiki/Amminoacido_N-acetiltransferasi" title="Amminoacido N-acetiltransferasi – Italian" lang="it" hreflang="it" data-title="Amminoacido N-acetiltransferasi" data-language-autonym="Italiano" data-language-local-name="Italian" class="interlanguage-link-target"><span>Italiano</span></a></li><li class="interlanguage-link interwiki-pt mw-list-item"><a href="https://pt.wikipedia.org/wiki/N-acetilglutamato_sintase" title="N-acetilglutamato sintase – Portuguese" lang="pt" 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.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"><i>N</i>-Acetylglutamate synthase</th></tr><tr><td colspan="2" class="infobox-image"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:4kzt.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/2/26/4kzt.jpg/220px-4kzt.jpg" decoding="async" width="220" height="175" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/2/26/4kzt.jpg/330px-4kzt.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/2/26/4kzt.jpg/440px-4kzt.jpg 2x" data-file-width="1000" data-file-height="797" /></a></span><div class="infobox-caption"><i>N</i>-Acetylglutamate synthase/kinase tetramer, <i>Maricaulis maris</i></div></td></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">NAGS</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=162417&amp;rn=1">162417</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:17996">17996</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/608300">608300</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/NM_153006">NM_153006</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Swiss-Prot" class="mw-redirect" title="Swiss-Prot">UniProt</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprot/Q8N159">Q8N159</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/Enzyme_Commission_number" title="Enzyme Commission number">EC number</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.genome.jp/dbget-bin/www_bget?enzyme+2.3.1.1">2.3.1.1</a></td></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_17_(human)" class="mw-redirect" title="Chromosome 17 (human)">Chr. 17</a> <i><a rel="nofollow" class="external text" href="https://omim.org/search/?index=geneMap&amp;search=17q21.31">q21.31</a></i></td></tr><tr><td colspan="2" class="infobox-full-data" style="background-color: #eee"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1257001546"><table class="infobox mw-collapsible mw-collapsed" style="float:none; clear:none; margin:0; border-width:0; border-collapse:collapse; text-align:left; width:100%"><tbody><tr><th colspan="2" class="infobox-header" style="background-color: #ddd">Search for</th></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3; border:#fafafa 2px solid; border-width:3px 2px 0 0;">Structures</th><td class="infobox-data" style="background-color: #eee; border:#fafafa 2px solid; border-width:3px 0 0 2px;"><a rel="nofollow" class="external text" href="https://swissmodel.expasy.org/repository/uniprot/Q8N159">Swiss-model</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3; border:#fafafa 2px solid; border-width:3px 2px 0 0;">Domains</th><td class="infobox-data" style="background-color: #eee; border:#fafafa 2px solid; border-width:3px 0 0 2px;"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/protein/Q8N159">InterPro</a></td></tr></tbody></table></td></tr></tbody></table> <p><b><i>N</i>-Acetylglutamate synthase</b> (<b>NAGS</b>) is an <a href="/wiki/Enzyme" title="Enzyme">enzyme</a> that catalyses the production of <a href="/wiki/N-Acetylglutamate" class="mw-redirect" title="N-Acetylglutamate"><i>N</i>-acetylglutamate</a> (NAG) from <a href="/wiki/Glutamate" class="mw-redirect" title="Glutamate">glutamate</a> and <a href="/wiki/Acetyl-CoA" title="Acetyl-CoA">acetyl-CoA</a>. </p><p>Put simply NAGS catalyzes the following reaction: </p> <dl><dd>acetyl-CoA + <small>L</small>-glutamate → CoA + <i>N</i>-acetyl-<small>L</small>-glutamate</dd></dl> <p>NAGS, a member of the <a href="/wiki/N-acetyltransferase" title="N-acetyltransferase"><i>N</i>-acetyltransferase</a> family of enzymes, is present in both <a href="/wiki/Prokaryotes" class="mw-redirect" title="Prokaryotes">prokaryotes</a> and <a href="/wiki/Eukaryotes" class="mw-redirect" title="Eukaryotes">eukaryotes</a>, although its role and structure differ widely depending on the species. NAG can be used in the production of <a href="/wiki/Ornithine" title="Ornithine">ornithine</a> and <a href="/wiki/Arginine" title="Arginine">arginine</a>, two important <a href="/wiki/Amino_acids" class="mw-redirect" title="Amino acids">amino acids</a>, or as an <a href="/wiki/Allosteric" class="mw-redirect" title="Allosteric">allosteric</a> <a href="/wiki/Cofactor_(biochemistry)" title="Cofactor (biochemistry)">cofactor</a> for <a href="/wiki/Carbamoyl_phosphate_synthase" class="mw-redirect" title="Carbamoyl phosphate synthase">carbamoyl phosphate synthase</a> (CPS1). In mammals, NAGS is expressed primarily in the <a href="/wiki/Liver" title="Liver">liver</a> and <a href="/wiki/Small_intestine" title="Small intestine">small intestine</a>, and is localized to the <a href="/wiki/Mitochondrial_matrix" title="Mitochondrial matrix">mitochondrial matrix</a>.<sup id="cite_ref-Meijer_1985_1-0" class="reference"><a href="#cite_note-Meijer_1985-1"><span class="cite-bracket">&#91;</span>1<span class="cite-bracket">&#93;</span></a></sup> </p> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:N-Acetylglutamate_Synthase_Chemical_Equation.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/0/0f/N-Acetylglutamate_Synthase_Chemical_Equation.png/220px-N-Acetylglutamate_Synthase_Chemical_Equation.png" decoding="async" width="220" height="77" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/0/0f/N-Acetylglutamate_Synthase_Chemical_Equation.png/330px-N-Acetylglutamate_Synthase_Chemical_Equation.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/0/0f/N-Acetylglutamate_Synthase_Chemical_Equation.png/440px-N-Acetylglutamate_Synthase_Chemical_Equation.png 2x" data-file-width="6126" data-file-height="2156" /></a><figcaption>Overall reaction scheme for <i>N</i>-acetylglutamate (NAG) synthesis via <i>N</i>-acetylglutamate synthase (NAGS)</figcaption></figure> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Biological_function">Biological function</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=N-Acetylglutamate_synthase&amp;action=edit&amp;section=1" title="Edit section: Biological function"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Most prokaryotes (<a href="/wiki/Bacteria" title="Bacteria">bacteria</a>) and lower eukaryotes (<a href="/wiki/Fungus" title="Fungus">fungus</a>, <a href="/wiki/Green_algae" title="Green algae">green algae</a>, <a href="/wiki/Plants" class="mw-redirect" title="Plants">plants</a>, and so on) produce NAG through ornithine acetyltransferase (OAT), which is part of a ‘cyclic’ ornithine production pathway. NAGS is therefore used in a supportive role, replenishing NAG reserves as required. In some plants and bacteria, however, NAGS catalyzes the first step in a ‘linear’ arginine production pathway.<sup id="cite_ref-Cunin_1986_2-0" class="reference"><a href="#cite_note-Cunin_1986-2"><span class="cite-bracket">&#91;</span>2<span class="cite-bracket">&#93;</span></a></sup> </p><p>The protein sequences of NAGS between prokaryotes, lower eukaryotes and higher eukaryotes have shown a remarkable lack of similarity. Sequence identity between prokaryotic and eukaryotic NAGS is largely &lt;30%,<sup id="cite_ref-pmid8939437_3-0" class="reference"><a href="#cite_note-pmid8939437-3"><span class="cite-bracket">&#91;</span>3<span class="cite-bracket">&#93;</span></a></sup> while sequence identity between lower and higher eukaryotes is ~20%.<sup id="cite_ref-Caldovic_2010_4-0" class="reference"><a href="#cite_note-Caldovic_2010-4"><span class="cite-bracket">&#91;</span>4<span class="cite-bracket">&#93;</span></a></sup> </p><p>Enzyme activity of NAGS is modulated by <a href="/wiki/L-arginine" class="mw-redirect" title="L-arginine"><small>L</small>-arginine</a>, which acts as an <a href="/wiki/Enzyme_inhibitor" title="Enzyme inhibitor">inhibitor</a> in plant and bacterial NAGS, but an effector in <a href="/wiki/Vertebrates" class="mw-redirect" title="Vertebrates">vertebrates</a>.<sup id="cite_ref-Cybis_1975_5-0" class="reference"><a href="#cite_note-Cybis_1975-5"><span class="cite-bracket">&#91;</span>5<span class="cite-bracket">&#93;</span></a></sup><sup id="cite_ref-pmid6885773_6-0" class="reference"><a href="#cite_note-pmid6885773-6"><span class="cite-bracket">&#91;</span>6<span class="cite-bracket">&#93;</span></a></sup> While the role of arginine as an inhibitor of NAG in ornithine and arginine synthesis is well understood, there is some controversy as to the role of NAG in the <a href="/wiki/Urea_cycle" title="Urea cycle">urea cycle</a>.<sup id="cite_ref-Meijer_1984_7-0" class="reference"><a href="#cite_note-Meijer_1984-7"><span class="cite-bracket">&#91;</span>7<span class="cite-bracket">&#93;</span></a></sup><sup id="cite_ref-pmid6721845_8-0" class="reference"><a href="#cite_note-pmid6721845-8"><span class="cite-bracket">&#91;</span>8<span class="cite-bracket">&#93;</span></a></sup> The currently accepted role of NAG in vertebrates is as an essential allosteric cofactor for CPS1, and therefore it acts as the primary controller of <a href="/wiki/Flux" title="Flux">flux</a> through the urea cycle. In this role, <a href="/wiki/Feedback_regulation" class="mw-redirect" title="Feedback regulation">feedback regulation</a> from arginine would act to signal NAGS that <a href="/wiki/Ammonia" title="Ammonia">ammonia</a> is plentiful within the cell, and needs to be removed, accelerating NAGS function. As it stands, the evolutionary journey of NAGS from essential synthetic enzyme to primary urea cycle controller is yet to be fully understood.<sup id="cite_ref-Caldovic_2003b_9-0" class="reference"><a href="#cite_note-Caldovic_2003b-9"><span class="cite-bracket">&#91;</span>9<span class="cite-bracket">&#93;</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Mechanism">Mechanism</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=N-Acetylglutamate_synthase&amp;action=edit&amp;section=2" title="Edit section: Mechanism"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:Reaction_mechanism_for_N-acetylglutamate_synthase.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/Reaction_mechanism_for_N-acetylglutamate_synthase.png/220px-Reaction_mechanism_for_N-acetylglutamate_synthase.png" decoding="async" width="220" height="104" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/Reaction_mechanism_for_N-acetylglutamate_synthase.png/330px-Reaction_mechanism_for_N-acetylglutamate_synthase.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/a/a2/Reaction_mechanism_for_N-acetylglutamate_synthase.png/440px-Reaction_mechanism_for_N-acetylglutamate_synthase.png 2x" data-file-width="8076" data-file-height="3831" /></a><figcaption>A simplified reaction mechanism for <i>N</i>-acetylglutamate synthase (NAGS)</figcaption></figure> <p>Two <a href="/wiki/Reaction_mechanism" title="Reaction mechanism">mechanisms</a> for <i>N</i>-acetyltransferase function have been proposed: a two-step, ping-pong mechanism involving transfer of the relevant <a href="/wiki/Acetyl_group" title="Acetyl group">acetyl group</a> to an activated <a href="/wiki/Cysteine" title="Cysteine">cysteine</a> residue<sup id="cite_ref-Wong_1983_10-0" class="reference"><a href="#cite_note-Wong_1983-10"><span class="cite-bracket">&#91;</span>10<span class="cite-bracket">&#93;</span></a></sup> and a one-step mechanism through direct attack of the <a href="/wiki/Amino" class="mw-redirect" title="Amino">amino</a> <a href="/wiki/Nitrogen" title="Nitrogen">nitrogen</a> on the <a href="/wiki/Carbonyl" class="mw-redirect" title="Carbonyl">carbonyl</a> group.<sup id="cite_ref-Dyda_2000_11-0" class="reference"><a href="#cite_note-Dyda_2000-11"><span class="cite-bracket">&#91;</span>11<span class="cite-bracket">&#93;</span></a></sup> Studies conducted using NAGS derived from <i><a href="/wiki/Neisseria_gonorrhoeae" title="Neisseria gonorrhoeae">Neisseria gonorrhoeae</a></i> suggest that NAGS proceeds through the previously described one-step mechanism.<sup id="cite_ref-Shi_2008_12-0" class="reference"><a href="#cite_note-Shi_2008-12"><span class="cite-bracket">&#91;</span>12<span class="cite-bracket">&#93;</span></a></sup> In this proposal, the carbonyl group of acetyl-CoA is attacked directly by the α-amino nitrogen of glutamate. This mechanism is supported by the activation of the carbonyl through <a href="/wiki/Hydrogen_bond" title="Hydrogen bond">hydrogen bond</a> <a href="/wiki/Polarizability" title="Polarizability">polarization</a>, as well as the absence of a suitable cysteine within the active site to act as an intermediate acceptor of the acetyl group.<sup id="cite_ref-Min_2009_13-0" class="reference"><a href="#cite_note-Min_2009-13"><span class="cite-bracket">&#91;</span>13<span class="cite-bracket">&#93;</span></a></sup><sup id="cite_ref-Morizono_2004_14-0" class="reference"><a href="#cite_note-Morizono_2004-14"><span class="cite-bracket">&#91;</span>14<span class="cite-bracket">&#93;</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=N-Acetylglutamate_synthase&amp;action=edit&amp;section=3" title="Edit section: Clinical significance"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Inactivity of NAGS results in <a href="/wiki/N-acetylglutamate_synthase_deficiency" class="mw-redirect" title="N-acetylglutamate synthase deficiency"><i>N</i>-acetylglutamate synthase deficiency</a>, a form of <a href="/wiki/Hyperammonemia" title="Hyperammonemia">hyperammonemia</a>.<sup id="cite_ref-Caldovic_2003_15-0" class="reference"><a href="#cite_note-Caldovic_2003-15"><span class="cite-bracket">&#91;</span>15<span class="cite-bracket">&#93;</span></a></sup> In many vertebrates, <i>N</i>-acetylglutamate is an essential allosteric cofactor of CPS1, the enzyme that catalyzes the first step of the urea cycle.<sup id="cite_ref-McCudden_1996_16-0" class="reference"><a href="#cite_note-McCudden_1996-16"><span class="cite-bracket">&#91;</span>16<span class="cite-bracket">&#93;</span></a></sup> Without NAG stimulation, CPS1 cannot convert ammonia to <a href="/wiki/Carbamoyl_phosphate" title="Carbamoyl phosphate">carbamoyl phosphate</a>, resulting in toxic ammonia accumulation.<sup id="cite_ref-Caldovic_2004_17-0" class="reference"><a href="#cite_note-Caldovic_2004-17"><span class="cite-bracket">&#91;</span>17<span class="cite-bracket">&#93;</span></a></sup> <a href="/w/index.php?title=Carbamoyl_glutamate&amp;action=edit&amp;redlink=1" class="new" title="Carbamoyl glutamate (page does not exist)">Carbamoyl glutamate</a> has shown promise as a possible treatment for NAGS deficiency.<sup id="cite_ref-Caldovic_2003_15-1" class="reference"><a href="#cite_note-Caldovic_2003-15"><span class="cite-bracket">&#91;</span>15<span class="cite-bracket">&#93;</span></a></sup> This is suspected to be a result of the structural similarities between NAG and carbamoyl glutamate, which allows carbamoyl glutamate to act as an effective <a href="/wiki/Agonist" title="Agonist">agonist</a> for CPS1.<sup id="cite_ref-Morizono_2004_14-1" class="reference"><a href="#cite_note-Morizono_2004-14"><span class="cite-bracket">&#91;</span>14<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=N-Acetylglutamate_synthase&amp;action=edit&amp;section=4" 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-Meijer_1985-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-Meijer_1985_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="CITEREFMeijerLofRamosVerhoeven1985" class="citation journal cs1">Meijer AJ, Lof C, Ramos IC, Verhoeven AJ (April 1985). <a rel="nofollow" class="external text" href="https://doi.org/10.1111%2Fj.1432-1033.1985.tb08824.x">"Control of ureogenesis"</a>. <i>European Journal of Biochemistry</i>. <b>148</b> (1): 189–96. <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/10.1111%2Fj.1432-1033.1985.tb08824.x">10.1111/j.1432-1033.1985.tb08824.x</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/3979393">3979393</a>.</cite><span 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class="mw-cite-backlink"><b><a href="#cite_ref-Min_2009_13-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFMinJinCaldovicMorizono2009" class="citation journal cs1">Min L, Jin Z, Caldovic L, Morizono H, Allewell NM, Tuchman M, Shi D (February 2009). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2643497">"Mechanism of allosteric inhibition of <i>N</i>-acetyl-L-glutamate synthase by L-arginine"</a>. <i>The Journal of Biological Chemistry</i>. <b>284</b> (8): 4873–80. <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/10.1074%2Fjbc.M805348200">10.1074/jbc.M805348200</a></span>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a>&#160;<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2643497">2643497</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/19095660">19095660</a>.</cite><span title="ctx_ver=Z39.88-2004&amp;rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&amp;rft.genre=article&amp;rft.jtitle=The+Journal+of+Biological+Chemistry&amp;rft.atitle=Mechanism+of+allosteric+inhibition+of+N-acetyl-L-glutamate+synthase+by+L-arginine&amp;rft.volume=284&amp;rft.issue=8&amp;rft.pages=4873-80&amp;rft.date=2009-02&amp;rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC2643497%23id-name%3DPMC&amp;rft_id=info%3Apmid%2F19095660&amp;rft_id=info%3Adoi%2F10.1074%2Fjbc.M805348200&amp;rft.aulast=Min&amp;rft.aufirst=L&amp;rft.au=Jin%2C+Z&amp;rft.au=Caldovic%2C+L&amp;rft.au=Morizono%2C+H&amp;rft.au=Allewell%2C+NM&amp;rft.au=Tuchman%2C+M&amp;rft.au=Shi%2C+D&amp;rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC2643497&amp;rfr_id=info%3Asid%2Fen.wikipedia.org%3AN-Acetylglutamate+synthase" class="Z3988"></span></span> </li> <li id="cite_note-Morizono_2004-14"><span class="mw-cite-backlink">^ <a href="#cite_ref-Morizono_2004_14-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Morizono_2004_14-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFMorizonoCaldovicShiTuchman2004" class="citation journal cs1">Morizono H, Caldovic L, Shi D, Tuchman M (April 2004). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3031861">"Mammalian <i>N</i>-acetylglutamate synthase"</a>. <i>Molecular Genetics and Metabolism</i>. <b>81</b> (Suppl 1): S4–11. <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.ymgme.2003.10.017">10.1016/j.ymgme.2003.10.017</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a>&#160;<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3031861">3031861</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/15050968">15050968</a>.</cite><span title="ctx_ver=Z39.88-2004&amp;rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&amp;rft.genre=article&amp;rft.jtitle=Molecular+Genetics+and+Metabolism&amp;rft.atitle=Mammalian+N-acetylglutamate+synthase&amp;rft.volume=81&amp;rft.issue=Suppl+1&amp;rft.pages=S4-11&amp;rft.date=2004-04&amp;rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3031861%23id-name%3DPMC&amp;rft_id=info%3Apmid%2F15050968&amp;rft_id=info%3Adoi%2F10.1016%2Fj.ymgme.2003.10.017&amp;rft.aulast=Morizono&amp;rft.aufirst=H&amp;rft.au=Caldovic%2C+L&amp;rft.au=Shi%2C+D&amp;rft.au=Tuchman%2C+M&amp;rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3031861&amp;rfr_id=info%3Asid%2Fen.wikipedia.org%3AN-Acetylglutamate+synthase" class="Z3988"></span></span> </li> <li id="cite_note-Caldovic_2003-15"><span class="mw-cite-backlink">^ <a href="#cite_ref-Caldovic_2003_15-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Caldovic_2003_15-1"><sup><i><b>b</b></i></sup></a></span> <span 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"Null mutations in the <i>N</i>-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia". <i>Human Genetics</i>. <b>112</b> (4): 364–8. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1007%2Fs00439-003-0909-5">10.1007/s00439-003-0909-5</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/12594532">12594532</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:27479847">27479847</a>.</cite><span title="ctx_ver=Z39.88-2004&amp;rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&amp;rft.genre=article&amp;rft.jtitle=Human+Genetics&amp;rft.atitle=Null+mutations+in+the+N-acetylglutamate+synthase+gene+associated+with+acute+neonatal+disease+and+hyperammonemia&amp;rft.volume=112&amp;rft.issue=4&amp;rft.pages=364-8&amp;rft.date=2003-04&amp;rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A27479847%23id-name%3DS2CID&amp;rft_id=info%3Apmid%2F12594532&amp;rft_id=info%3Adoi%2F10.1007%2Fs00439-003-0909-5&amp;rft.aulast=Caldovic&amp;rft.aufirst=L&amp;rft.au=Morizono%2C+H&amp;rft.au=Panglao%2C+MG&amp;rft.au=Cheng%2C+SF&amp;rft.au=Packman%2C+S&amp;rft.au=Tuchman%2C+M&amp;rfr_id=info%3Asid%2Fen.wikipedia.org%3AN-Acetylglutamate+synthase" class="Z3988"></span></span> </li> <li id="cite_note-McCudden_1996-16"><span class="mw-cite-backlink"><b><a href="#cite_ref-McCudden_1996_16-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFMcCuddenPowers-Lee1996" class="citation journal cs1">McCudden CR, Powers-Lee SG (July 1996). <a rel="nofollow" class="external text" href="https://doi.org/10.1074%2Fjbc.271.30.18285">"Required allosteric effector site for <i>N</i>-acetylglutamate on carbamoyl-phosphate synthetase I"</a>. <i>The Journal of Biological Chemistry</i>. <b>271</b> (30): 18285–94. <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/10.1074%2Fjbc.271.30.18285">10.1074/jbc.271.30.18285</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/8663466">8663466</a>.</cite><span title="ctx_ver=Z39.88-2004&amp;rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&amp;rft.genre=article&amp;rft.jtitle=The+Journal+of+Biological+Chemistry&amp;rft.atitle=Required+allosteric+effector+site+for+N-acetylglutamate+on+carbamoyl-phosphate+synthetase+I&amp;rft.volume=271&amp;rft.issue=30&amp;rft.pages=18285-94&amp;rft.date=1996-07&amp;rft_id=info%3Adoi%2F10.1074%2Fjbc.271.30.18285&amp;rft_id=info%3Apmid%2F8663466&amp;rft.aulast=McCudden&amp;rft.aufirst=CR&amp;rft.au=Powers-Lee%2C+SG&amp;rft_id=https%3A%2F%2Fdoi.org%2F10.1074%252Fjbc.271.30.18285&amp;rfr_id=info%3Asid%2Fen.wikipedia.org%3AN-Acetylglutamate+synthase" class="Z3988"></span></span> </li> <li id="cite_note-Caldovic_2004-17"><span class="mw-cite-backlink"><b><a href="#cite_ref-Caldovic_2004_17-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFCaldovicMorizonoDaikhinNissim2004" class="citation journal cs1">Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M (October 2004). "Restoration of ureagenesis in <i>N</i>-acetylglutamate synthase deficiency by <i>N</i>-carbamylglutamate". <i>The Journal of Pediatrics</i>. <b>145</b> (4): 552–4. <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.jpeds.2004.06.047">10.1016/j.jpeds.2004.06.047</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/15480384">15480384</a>.</cite><span title="ctx_ver=Z39.88-2004&amp;rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&amp;rft.genre=article&amp;rft.jtitle=The+Journal+of+Pediatrics&amp;rft.atitle=Restoration+of+ureagenesis+in+N-acetylglutamate+synthase+deficiency+by+N-carbamylglutamate&amp;rft.volume=145&amp;rft.issue=4&amp;rft.pages=552-4&amp;rft.date=2004-10&amp;rft_id=info%3Adoi%2F10.1016%2Fj.jpeds.2004.06.047&amp;rft_id=info%3Apmid%2F15480384&amp;rft.aulast=Caldovic&amp;rft.aufirst=L&amp;rft.au=Morizono%2C+H&amp;rft.au=Daikhin%2C+Y&amp;rft.au=Nissim%2C+I&amp;rft.au=McCarter%2C+RJ&amp;rft.au=Yudkoff%2C+M&amp;rft.au=Tuchman%2C+M&amp;rfr_id=info%3Asid%2Fen.wikipedia.org%3AN-Acetylglutamate+synthase" class="Z3988"></span></span> </li> </ol></div></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=N-Acetylglutamate_synthase&amp;action=edit&amp;section=5" title="Edit section: External 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template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Urea_cycle_enzymes" title="Template talk:Urea cycle enzymes"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Urea_cycle_enzymes" title="Special:EditPage/Template:Urea cycle enzymes"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Metabolism:_amino_acid_metabolism_-_urea_cycle_enzymes" style="font-size:114%;margin:0 4em"><a href="/wiki/Metabolism" title="Metabolism">Metabolism</a>: <a href="/wiki/Protein_metabolism" title="Protein metabolism">amino acid metabolism</a> - <a href="/wiki/Urea_cycle" title="Urea cycle">urea cycle</a> <a href="/wiki/Enzyme" title="Enzyme">enzymes</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%">Main cycle</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/Mitochondrial_matrix" title="Mitochondrial matrix">mitochondrial matrix</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/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></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Cytosol" title="Cytosol">cytosol</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/Argininosuccinate_synthase" title="Argininosuccinate synthase">Argininosuccinate synthetase</a></li> <li><a href="/wiki/Argininosuccinate_lyase" title="Argininosuccinate lyase">Argininosuccinate lyase</a></li> <li><a href="/wiki/Arginase" title="Arginase">Arginase</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Regulatory/transport</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 class="mw-selflink selflink">N-Acetylglutamate synthase</a></li> <li><a href="/wiki/Ornithine_translocase" title="Ornithine translocase">Ornithine translocase</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="Mitochondrial_proteins" 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:Mitochondrial_proteins" title="Template:Mitochondrial proteins"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Mitochondrial_proteins" title="Template talk:Mitochondrial proteins"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Mitochondrial_proteins" title="Special:EditPage/Template:Mitochondrial proteins"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Mitochondrial_proteins" style="font-size:114%;margin:0 4em"><a href="/wiki/Mitochondrion" title="Mitochondrion">Mitochondrial</a> proteins</div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Outer_mitochondrial_membrane" class="mw-redirect" title="Outer mitochondrial membrane">Outer 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/Fatty_acid_degradation" title="Fatty acid degradation">fatty acid degradation</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/Carnitine_palmitoyltransferase_I" title="Carnitine palmitoyltransferase I">Carnitine palmitoyltransferase I</a></li> <li><a href="/wiki/Long-chain-fatty-acid%E2%80%94CoA_ligase" title="Long-chain-fatty-acid—CoA ligase">Long-chain-fatty-acid—CoA ligase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i><a href="/wiki/Tryptophan" title="Tryptophan">tryptophan</a> metabolism</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/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 class="mw-selflink selflink">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 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> <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="Transferases:_acyltransferases_(EC_2.3)" 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:Acyltransferases" title="Template:Acyltransferases"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Acyltransferases" title="Template talk:Acyltransferases"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Acyltransferases" title="Special:EditPage/Template:Acyltransferases"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Transferases:_acyltransferases_(EC_2.3)" style="font-size:114%;margin:0 4em"><a href="/wiki/Transferase" title="Transferase">Transferases</a>: <a href="/wiki/Acyltransferase" title="Acyltransferase">acyltransferases</a> (<a href="/wiki/Enzyme_Commission_number" title="Enzyme Commission number">EC</a> 2.3)</div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/List_of_EC_numbers_(EC_2)#EC_2.3.1:_Transferring_groups_other_than_amino-acyl_groups" title="List of EC numbers (EC 2)">2.3.1</a>: other than amino-acyl groups</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/Acetyltransferase" title="Acetyltransferase">acetyltransferases</a>:</i> <a href="/wiki/Thiolase" title="Thiolase">Acetyl-Coenzyme A acetyltransferase</a></li> <li><a class="mw-selflink selflink">N-Acetylglutamate synthase</a></li> <li><a href="/wiki/Choline_acetyltransferase" title="Choline acetyltransferase">Choline acetyltransferase</a></li> <li><a href="/wiki/Dihydrolipoyl_transacetylase" title="Dihydrolipoyl transacetylase">Dihydrolipoyl transacetylase</a></li> <li><a href="/wiki/Acetyl-CoA_C-acyltransferase" class="mw-redirect" title="Acetyl-CoA C-acyltransferase">Acetyl-CoA C-acyltransferase</a></li> <li><a href="/wiki/Beta-galactoside_transacetylase" class="mw-redirect" title="Beta-galactoside transacetylase">Beta-galactoside transacetylase</a></li> <li><a href="/wiki/Chloramphenicol_acetyltransferase" title="Chloramphenicol acetyltransferase">Chloramphenicol acetyltransferase</a></li> <li><a href="/wiki/N-acetyltransferase" title="N-acetyltransferase">N-acetyltransferase</a> <ul><li><a href="/wiki/Serotonin_N-acetyl_transferase" class="mw-redirect" title="Serotonin N-acetyl transferase">Serotonin N-acetyl transferase</a></li> <li><a href="/wiki/HGSNAT" title="HGSNAT">HGSNAT</a></li> <li><a href="/wiki/ARD1A" class="mw-redirect" title="ARD1A">ARD1A</a></li></ul></li> <li><a href="/wiki/Histone_acetyltransferase" title="Histone acetyltransferase">Histone acetyltransferase</a> <ul><li><a href="/wiki/P300-CBP_coactivator_family" title="P300-CBP coactivator family">P300/CBP</a></li> <li><a href="/wiki/NAT2" class="mw-redirect" title="NAT2">NAT2</a></li></ul></li></ul> <ul><li><i><a href="/wiki/Palmitoyltransferase" class="mw-redirect" title="Palmitoyltransferase">palmitoyltransferases</a>:</i> <a href="/wiki/Carnitine_O-palmitoyltransferase" title="Carnitine O-palmitoyltransferase">Carnitine O-palmitoyltransferase</a> <ul><li><a href="/wiki/Carnitine_palmitoyltransferase_I" title="Carnitine palmitoyltransferase I">CPT1</a></li> <li><a href="/wiki/Carnitine_palmitoyltransferase_II" title="Carnitine palmitoyltransferase II">CPT2</a></li></ul></li> <li><a href="/wiki/Serine_C-palmitoyltransferase" title="Serine C-palmitoyltransferase">Serine C-palmitoyltransferase</a> <ul><li><a href="/wiki/SPTLC1" title="SPTLC1">SPTLC1</a></li> <li><a href="/wiki/SPTLC2" title="SPTLC2">SPTLC2</a></li></ul></li></ul> <ul><li><i>other:</i> <a href="/wiki/Acyltransferase_like_2" title="Acyltransferase like 2">Acyltransferase like 2</a></li> <li><a href="/wiki/Aminolevulinic_acid_synthase" title="Aminolevulinic acid synthase">Aminolevulinic acid synthase</a></li> <li><a href="/wiki/Beta-ketoacyl-ACP_synthase" title="Beta-ketoacyl-ACP synthase">Beta-ketoacyl-ACP synthase</a></li> <li><a href="/wiki/Glyceronephosphate_O-acyltransferase" title="Glyceronephosphate O-acyltransferase">Glyceronephosphate O-acyltransferase</a></li> <li><a href="/wiki/Lecithin%E2%80%94cholesterol_acyltransferase" class="mw-redirect" title="Lecithin—cholesterol acyltransferase">Lecithin—cholesterol acyltransferase </a></li></ul> <ul><li><a href="/wiki/Glycerol-3-phosphate_O-acyltransferase" title="Glycerol-3-phosphate O-acyltransferase">Glycerol-3-phosphate O-acyltransferase</a></li> <li><a href="/wiki/1-acylglycerol-3-phosphate_O-acyltransferase" title="1-acylglycerol-3-phosphate O-acyltransferase">1-acylglycerol-3-phosphate O-acyltransferase</a></li> <li><a href="/wiki/2-acylglycerol-3-phosphate_O-acyltransferase" title="2-acylglycerol-3-phosphate O-acyltransferase">2-acylglycerol-3-phosphate O-acyltransferase</a></li> <li><a href="/wiki/ABHD5" title="ABHD5">ABHD5</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/List_of_EC_numbers_(EC_2)#EC_2.3.2:_Aminoacyltransferases" title="List of EC numbers (EC 2)">2.3.2</a>: <a href="/wiki/Aminoacyltransferase" title="Aminoacyltransferase">Aminoacyltransferases</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/Gamma-glutamyl_transpeptidase" class="mw-redirect" title="Gamma-glutamyl transpeptidase">Gamma-glutamyl transpeptidase</a></li> <li><a href="/wiki/Peptidyl_transferase" class="mw-redirect" title="Peptidyl transferase">Peptidyl transferase</a></li> <li><a href="/wiki/Transglutaminase" title="Transglutaminase">Transglutaminase</a> <ul><li><a href="/wiki/Tissue_transglutaminase" title="Tissue transglutaminase">Tissue transglutaminase</a></li> <li><a href="/wiki/Keratinocyte_transglutaminase" title="Keratinocyte transglutaminase">Keratinocyte transglutaminase</a></li> <li><a href="/wiki/Factor_XIII" title="Factor XIII">Factor XIII</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/List_of_EC_numbers_(EC_2)#EC_2.3.3:_Acyl_groups_converted_into_alkyl_on_transfer" title="List of EC numbers (EC 2)">2.3.3</a>: converted into alkyl on transfer</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/Citrate_synthase" title="Citrate synthase">Citrate synthase</a></li> <li><a href="/wiki/Decylcitrate_synthase" title="Decylcitrate synthase">Decylcitrate synthase</a></li> <li><a href="/wiki/Citrate_(Re)-synthase" title="Citrate (Re)-synthase">Citrate (Re)-synthase</a></li> <li><a href="/wiki/Decylhomocitrate_synthase" title="Decylhomocitrate synthase">Decylhomocitrate synthase</a></li> <li><a href="/wiki/2-methylcitrate_synthase" title="2-methylcitrate synthase">2-methylcitrate synthase</a></li> <li><a href="/wiki/2-ethylmalate_synthase" title="2-ethylmalate synthase">2-ethylmalate synthase</a></li> <li><a href="/wiki/3-ethylmalate_synthase" title="3-ethylmalate synthase">3-ethylmalate synthase</a></li> <li><a href="/wiki/ATP_citrate_lyase" class="mw-redirect" title="ATP citrate lyase">ATP citrate lyase</a></li> <li><a href="/wiki/Malate_synthase" title="Malate synthase">Malate synthase</a></li> <li><a href="/wiki/Hydroxymethylglutaryl-CoA_synthase" title="Hydroxymethylglutaryl-CoA synthase">HMG-CoA synthase</a> <ul><li><a href="/wiki/HMGCS2" title="HMGCS2">HMGCS2</a></li></ul></li> <li><a href="/wiki/2-hydroxyglutarate_synthase" title="2-hydroxyglutarate synthase">2-hydroxyglutarate synthase</a></li> <li><a href="/wiki/3-propylmalate_synthase" title="3-propylmalate synthase">3-propylmalate synthase</a></li> <li><a href="/wiki/2-isopropylmalate_synthase" title="2-isopropylmalate synthase">2-isopropylmalate synthase</a></li> <li><a href="/wiki/Homocitrate_synthase" title="Homocitrate synthase">Homocitrate synthase</a></li> <li><a href="/wiki/Sulfoacetaldehyde_acetyltransferase" title="Sulfoacetaldehyde acetyltransferase">Sulfoacetaldehyde acetyltransferase</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="Enzymes" 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:Enzymes" title="Template:Enzymes"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Enzymes" title="Template talk:Enzymes"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Enzymes" title="Special:EditPage/Template:Enzymes"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Enzymes" style="font-size:114%;margin:0 4em"><a href="/wiki/Enzyme" title="Enzyme">Enzymes</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%">Activity</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/Active_site" title="Active site">Active site</a></li> <li><a href="/wiki/Binding_site" title="Binding site">Binding site</a></li> <li><a href="/wiki/Catalytic_triad" title="Catalytic triad">Catalytic triad</a></li> <li><a href="/wiki/Oxyanion_hole" title="Oxyanion hole">Oxyanion hole</a></li> <li><a href="/wiki/Enzyme_promiscuity" title="Enzyme promiscuity">Enzyme promiscuity</a></li> <li><a href="/wiki/Diffusion-limited_enzyme" title="Diffusion-limited enzyme">Diffusion-limited enzyme</a></li> <li><a href="/wiki/Cofactor_(biochemistry)" title="Cofactor (biochemistry)">Cofactor</a></li> <li><a href="/wiki/Enzyme_catalysis" title="Enzyme catalysis">Enzyme catalysis</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Regulation</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/Allosteric_regulation" title="Allosteric regulation">Allosteric regulation</a></li> <li><a href="/wiki/Cooperativity" title="Cooperativity">Cooperativity</a></li> <li><a href="/wiki/Enzyme_inhibitor" title="Enzyme inhibitor">Enzyme inhibitor</a></li> <li><a href="/wiki/Enzyme_activator" title="Enzyme activator">Enzyme activator</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Classification</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/Enzyme_Commission_number" title="Enzyme Commission number">EC number</a></li> <li><a href="/wiki/Protein_superfamily" title="Protein superfamily">Enzyme superfamily</a></li> <li><a href="/wiki/Protein_family" title="Protein family">Enzyme family</a></li> <li><a href="/wiki/List_of_enzymes" title="List of enzymes">List of enzymes</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Kinetics</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/Enzyme_kinetics" title="Enzyme kinetics">Enzyme kinetics</a></li> <li><a href="/wiki/Eadie%E2%80%93Hofstee_diagram" title="Eadie–Hofstee diagram">Eadie–Hofstee diagram</a></li> <li><a href="/wiki/Hanes%E2%80%93Woolf_plot" title="Hanes–Woolf plot">Hanes–Woolf plot</a></li> <li><a href="/wiki/Lineweaver%E2%80%93Burk_plot" title="Lineweaver–Burk plot">Lineweaver–Burk plot</a></li> <li><a href="/wiki/Michaelis%E2%80%93Menten_kinetics" title="Michaelis–Menten kinetics">Michaelis–Menten kinetics</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Types</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><b>EC1 <a href="/wiki/Oxidoreductase" title="Oxidoreductase">Oxidoreductases</a></b> (<a href="/wiki/List_of_EC_numbers_(EC_1)" title="List of EC numbers (EC 1)">list</a>)</li> <li><b>EC2 <a href="/wiki/Transferase" title="Transferase">Transferases</a></b> (<a href="/wiki/List_of_EC_numbers_(EC_2)" title="List of EC numbers (EC 2)">list</a>)</li> <li><b>EC3 <a href="/wiki/Hydrolase" title="Hydrolase">Hydrolases</a></b> (<a href="/wiki/List_of_EC_numbers_(EC_3)" title="List of EC numbers (EC 3)">list</a>)</li> <li><b>EC4 <a href="/wiki/Lyase" title="Lyase">Lyases</a></b> (<a href="/wiki/List_of_EC_numbers_(EC_4)" title="List of EC numbers (EC 4)">list</a>)</li> <li><b>EC5 <a href="/wiki/Isomerase" title="Isomerase">Isomerases</a></b> (<a href="/wiki/List_of_EC_numbers_(EC_5)" title="List of EC numbers (EC 5)">list</a>)</li> <li><b>EC6 <a href="/wiki/Ligase" title="Ligase">Ligases</a></b> (<a href="/wiki/List_of_EC_numbers_(EC_6)" title="List of EC numbers (EC 6)">list</a>)</li> <li><b>EC7 <a href="/wiki/Translocase" title="Translocase">Translocases</a></b> (<a href="/wiki/List_of_EC_numbers_(EC_7)" title="List of EC numbers (EC 7)">list</a>)</li></ul> </div></td></tr></tbody></table></div> <style data-mw-deduplicate="TemplateStyles:r1130092004">.mw-parser-output .portal-bar{font-size:88%;font-weight:bold;display:flex;justify-content:center;align-items:baseline}.mw-parser-output .portal-bar-bordered{padding:0 2em;background-color:#fdfdfd;border:1px solid #a2a9b1;clear:both;margin:1em auto 0}.mw-parser-output .portal-bar-related{font-size:100%;justify-content:flex-start}.mw-parser-output .portal-bar-unbordered{padding:0 1.7em;margin-left:0}.mw-parser-output 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