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dehidrogenaza" data-language-autonym="Srpskohrvatski / српскохрватски" data-language-local-name="Serbo-Croatian" class="interlanguage-link-target"><span>Srpskohrvatski / српскохрватски</span></a></li><li class="interlanguage-link interwiki-tt mw-list-item"><a href="https://tt.wikipedia.org/wiki/IVD" title="IVD – Tatar" lang="tt" hreflang="tt" data-title="IVD" data-language-autonym="Татарча / tatarça" data-language-local-name="Tatar" class="interlanguage-link-target"><span>Татарча / tatarça</span></a></li><li class="interlanguage-link interwiki-uk mw-list-item"><a href="https://uk.wikipedia.org/wiki/IVD" title="IVD – Ukrainian" lang="uk" hreflang="uk" data-title="IVD" data-language-autonym="Українська" data-language-local-name="Ukrainian" class="interlanguage-link-target"><span>Українська</span></a></li> </ul> <div class="after-portlet after-portlet-lang"><span class="wb-langlinks-edit wb-langlinks-link"><a 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<div id="contentSub"><div id="mw-content-subtitle"><span class="mw-redirectedfrom">(Redirected from <a href="/w/index.php?title=Isovaleryl_coenzyme_A_dehydrogenase&redirect=no" class="mw-redirect" title="Isovaleryl coenzyme A dehydrogenase">Isovaleryl coenzyme A dehydrogenase</a>)</span></div></div> <div id="mw-content-text" class="mw-body-content"><div class="mw-content-ltr mw-parser-output" lang="en" dir="ltr"><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">isovaleryl-CoA dehydrogenase</th></tr><tr><td colspan="2" class="infobox-image"><span typeof="mw:File"><a href="/wiki/File:1ivh.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/f2/1ivh.jpg/270px-1ivh.jpg" decoding="async" width="270" height="215" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/f2/1ivh.jpg/405px-1ivh.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/f2/1ivh.jpg/540px-1ivh.jpg 2x" data-file-width="1000" data-file-height="797" /></a></span><div class="infobox-caption">Isovaleryl-CoA dehydrogenase tetramer, Human</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"><a href="/wiki/Enzyme_Commission_number" title="Enzyme Commission number">EC no.</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.enzyme-database.org/query.php?ec=1.3.8.4">1.3.8.4</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/CAS_registry_number" class="mw-redirect" title="CAS registry number">CAS no.</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://commonchemistry.cas.org/detail?cas_rn=37274-61-6&title=">37274-61-6 </a></td></tr><tr><th colspan="2" class="infobox-header" style="background-color: #ddd">Databases</th></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/IntEnz" title="IntEnz">IntEnz</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/intenz/query?cmd=SearchEC&ec=1.3.8.4">IntEnz view</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/BRENDA" title="BRENDA">BRENDA</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="http://www.brenda-enzymes.org/enzyme.php?ecno=1.3.8.4">BRENDA entry</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/ExPASy" class="mw-redirect" title="ExPASy">ExPASy</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://enzyme.expasy.org/EC/1.3.8.4">NiceZyme view</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/KEGG" title="KEGG">KEGG</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+1.3.8.4">KEGG entry</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/MetaCyc" title="MetaCyc">MetaCyc</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://biocyc.org/META/substring-search?type=NIL&object=1.3.8.4">metabolic pathway</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/PRIAM_enzyme-specific_profiles" title="PRIAM enzyme-specific profiles">PRIAM</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="http://priam.prabi.fr/cgi-bin/PRIAM_profiles_CurrentRelease.pl?EC=1.3.8.4">profile</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Protein_Data_Bank" title="Protein Data Bank">PDB</a> structures</th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.rcsb.org/search?q=rcsb_polymer_entity.rcsb_ec_lineage.id:1.3.8.4">RCSB PDB</a> <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/pdbe/entry/search/index?ec_number:1.3.8.4">PDBe</a> <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/enzymes/GetPage.pl?ec_number=1.3.8.4">PDBsum</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Gene_Ontology" title="Gene Ontology">Gene Ontology</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="http://amigo.geneontology.org/amigo/term/GO:0008470">AmiGO </a> / <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/QuickGO/term/GO:0008470">QuickGO</a></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</th></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3; border:#fafafa 2px solid; border-width:3px 2px 0 0;"><a href="/wiki/PubMed_Central" title="PubMed Central">PMC</a></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.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&term=1.3.8.4%5BEC/RN%20Number%5D%20AND%20pubmed%20pmc%20local%5Bsb%5D">articles</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3; border:#fafafa 2px solid; border-width:3px 2px 0 0;"><a href="/wiki/PubMed" title="PubMed">PubMed</a></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.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&term=1.3.8.4%5BEC/RN%20Number%5D">articles</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3; border:#fafafa 2px solid; border-width:3px 2px 0 0;"><a href="/wiki/National_Center_for_Biotechnology_Information" title="National Center for Biotechnology Information">NCBI</a></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.ncbi.nlm.nih.gov/protein?term=1.3.8.4%5BEC/RN%20Number%5D">proteins</a></td></tr></tbody></table></td></tr></tbody></table> <p>In <a href="/wiki/Enzymology" class="mw-redirect" title="Enzymology">enzymology</a>, an <b>isovaleryl-CoA dehydrogenase</b> (<a href="/wiki/Enzyme_Commission_number" title="Enzyme Commission number">EC</a> <a rel="nofollow" class="external text" href="https://enzyme.expasy.org/EC/1.3.8.4">1.3.8.4</a>) is an <a href="/wiki/Enzyme" title="Enzyme">enzyme</a> that <a href="/wiki/Catalysis" title="Catalysis">catalyzes</a> the <a href="/wiki/Chemical_reaction" title="Chemical reaction">chemical reaction</a> </p> <dl><dd>3-methylbutanoyl-CoA + acceptor <span class="mwe-math-element"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \rightleftharpoons }"> <semantics> <mrow class="MJX-TeXAtom-ORD"> <mstyle displaystyle="true" scriptlevel="0"> <mo class="MJX-variant" stretchy="false">⇌</mo> </mstyle> </mrow> <annotation encoding="application/x-tex">{\displaystyle \rightleftharpoons }</annotation> </semantics> </math></span><img src="https://wikimedia.org/api/rest_v1/media/math/render/svg/1c37b981df851b9e54e489e017b1481e37d418f3" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -0.338ex; width:2.324ex; height:1.843ex;" alt="{\displaystyle \rightleftharpoons }"></span> 3-methylbut-2-enoyl-CoA + reduced acceptor</dd></dl> <p>Thus, the two <a href="/wiki/Substrate_(biochemistry)" class="mw-redirect" title="Substrate (biochemistry)">substrates</a> of this enzyme are <a href="/w/index.php?title=3-methylbutanoyl-CoA&action=edit&redlink=1" class="new" title="3-methylbutanoyl-CoA (page does not exist)">3-methylbutanoyl-CoA</a> and <a href="/wiki/Electron_acceptor" title="Electron acceptor">acceptor</a>, whereas its two <a href="/wiki/Product_(chemistry)" title="Product (chemistry)">products</a> are <a href="/w/index.php?title=3-methylbut-2-enoyl-CoA&action=edit&redlink=1" class="new" title="3-methylbut-2-enoyl-CoA (page does not exist)">3-methylbut-2-enoyl-CoA</a> and <a href="/wiki/Reduced_acceptor" class="mw-redirect" title="Reduced acceptor">reduced acceptor</a>. </p><p>This enzyme belongs to the family of <a href="/wiki/Oxidoreductase" title="Oxidoreductase">oxidoreductases</a>, specifically those acting on the CH-CH group of donor with other acceptors. The <a href="/wiki/List_of_enzymes" title="List of enzymes">systematic name</a> of this enzyme class is <b>3-methylbutanoyl-CoA:acceptor oxidoreductase</b>. Other names in common use include <b>isovaleryl-coenzyme A dehydrogenase</b>, <b>isovaleroyl-coenzyme A dehydrogenase</b>, and <b>3-methylbutanoyl-CoA:(acceptor) oxidoreductase</b>. This enzyme participates in <a href="/wiki/Valine,_leucine_and_isoleucine_degradation" class="mw-redirect" title="Valine, leucine and isoleucine degradation">valine, leucine and isoleucine degradation</a>. It employs one <a href="/wiki/Cofactor_(biochemistry)" title="Cofactor (biochemistry)">cofactor</a>, <a href="/wiki/Flavin_adenine_dinucleotide" title="Flavin adenine dinucleotide">FAD</a>. </p> <div class="mw-heading mw-heading2"><h2 id="Structural_studies">Structural studies</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Isovaleryl-CoA_dehydrogenase&action=edit&section=1" title="Edit section: Structural studies"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>As of late 2007, only one <a href="/wiki/Tertiary_structure" class="mw-redirect" title="Tertiary structure">structure</a> has been solved for this class of enzymes, with the <a href="/wiki/Protein_Data_Bank" title="Protein Data Bank">PDB</a> accession code <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=1IVH">1IVH</a>. It was created by a group containing K.A.Tiffany, D.L.Roberts, M.Wang, R.Paschke, A.-W.A.Mohsen, J.Vockley, and J.J.P.Kim. The structure was released on May 20, 1998.<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="CITEREFDoe" class="citation web cs1">Doe. <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=1IVH/">"PDBsum entry: 1ivh"</a><span class="reference-accessdate">. Retrieved <span class="nowrap">November 25,</span> 2019</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=unknown&rft.btitle=PDBsum+entry%3A+1ivh&rft.au=Doe&rft_id=https%3A%2F%2Fwww.ebi.ac.uk%2Fthornton-srv%2Fdatabases%2Fcgi-bin%2Fpdbsum%2FGetPage.pl%3Fpdbcode%3D1IVH%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIsovaleryl-CoA+dehydrogenase" class="Z3988"></span> </p> <div class="mw-heading mw-heading2"><h2 id="Leucine_metabolism">Leucine metabolism</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Isovaleryl-CoA_dehydrogenase&action=edit&section=2" title="Edit section: Leucine metabolism"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="skin-invert-image"> <table role="presentation" cellpadding="0" style="border-spacing:0; margin-left: auto; margin-right: auto; border: none;"> <tbody><tr> <td><div class="thumb center" style="margin: 1em auto;"> <div class="thumbinner" style="width:602px;"> <div style="clear: both; font-weight: bold; font-size: 106.4%; text-align: center; background-color: #F0F8FF;margin-bottom:3px;"><a href="/wiki/Leucine#Metabolism_in_humans" title="Leucine">Leucine metabolism in humans</a></div> <div style="position:relative; width:600px; height:436px; overflow:hidden; border:solid #ccc 1px; background-color:white;"> <div style="left:0px; top:0px; width:600px; position:absolute"> <span typeof="mw:File"><a href="https://commons.wikimedia.org/wiki/File:HMB_biosynthesis_and_metabolism_diagram_-_no_labels.svg" title="commons:File:HMB biosynthesis and metabolism diagram - no labels.svg"><img alt="Diagram of leucine, HMB, and isovaleryl-CoA metabolism in humans" src="//upload.wikimedia.org/wikipedia/commons/thumb/0/03/HMB_biosynthesis_and_metabolism_diagram_-_no_labels.svg/600px-HMB_biosynthesis_and_metabolism_diagram_-_no_labels.svg.png" decoding="async" width="600" height="436" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/0/03/HMB_biosynthesis_and_metabolism_diagram_-_no_labels.svg/900px-HMB_biosynthesis_and_metabolism_diagram_-_no_labels.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/0/03/HMB_biosynthesis_and_metabolism_diagram_-_no_labels.svg/1200px-HMB_biosynthesis_and_metabolism_diagram_-_no_labels.svg.png 2x" data-file-width="2126" data-file-height="1545" /></a></span> </div> <div style="text-align:center; font-size:12px; line-height:110%"> <div style="background-color:transparent; color:black"><div id="annotation_180x2" style="position:absolute; left:180px; top:2px; font-size:18px; font-size:18; line-height:20px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Leucine" title="Leucine"><span class="nowrap"><span class="smallcaps"><span style="font-variant: small-caps; text-transform: lowercase;">L</span></span>-Leucine</span></a></span></div> <div id="annotation_96x37" style="position:absolute; left:96px; top:37px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Branched-chain_amino_acid_aminotransferase" title="Branched-chain amino acid aminotransferase">Branched-chain amino<br />acid aminotransferase</a></span></div> <div id="annotation_240x30" style="position:absolute; left:240px; top:30px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/%CE%91-Ketoglutarate" class="mw-redirect" title="Α-Ketoglutarate">α-Ketoglutarate</a></span></div> <div id="annotation_243x61" style="position:absolute; left:243px; top:61px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Glutamate" class="mw-redirect" title="Glutamate">Glutamate</a></span></div> <div id="annotation_466x75" style="position:absolute; left:466px; top:75px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Glutamate" class="mw-redirect" title="Glutamate">Glutamate</a></span></div> <div id="annotation_383x30" style="position:absolute; left:383px; top:30px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Alanine" title="Alanine">Alanine</a></span></div> <div id="annotation_383x62" style="position:absolute; left:383px; top:62px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Pyruvate" class="mw-redirect" title="Pyruvate">Pyruvate</a></span></div> <div id="annotation_75x85" style="position:absolute; left:75px; top:85px; font-size:15px; font-size:15; line-height:17px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Skeletal_muscle" title="Skeletal muscle">Muscle</a>: <a href="/wiki/Alpha-Ketoisocaproic_acid" class="mw-redirect" title="Alpha-Ketoisocaproic acid">α-Ketoisocaproate</a> (α-KIC)</span></div> <div id="annotation_82x128" style="position:absolute; left:82px; top:128px; font-size:15px; font-size:15; line-height:17px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Liver" title="Liver">Liver</a>: <a href="/wiki/Alpha-Ketoisocaproic_acid" class="mw-redirect" title="Alpha-Ketoisocaproic acid">α-Ketoisocaproate</a> (α-KIC)</span></div> <div id="annotation_300x106" style="position:absolute; left:300px; top:106px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Branched-chain_%CE%B1-ketoacid_dehydrogenase" class="mw-redirect" title="Branched-chain α-ketoacid dehydrogenase">Branched-chain α-ketoacid<br />dehydrogenase</a> (<a href="/wiki/Mitochondria" class="mw-redirect" title="Mitochondria">mitochondria</a>)</span></div> <div id="annotation_93x156" style="position:absolute; left:93px; top:156px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/4-Hydroxyphenylpyruvate_dioxygenase" title="4-Hydroxyphenylpyruvate dioxygenase">KIC-dioxygenase</a><br />(<a href="/wiki/Cytosol" title="Cytosol">cytosol</a>)</span></div> <div id="annotation_465x126" style="position:absolute; left:465px; top:126px; font-size:15px; font-size:15; line-height:17px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Isovaleryl-CoA" title="Isovaleryl-CoA">Isovaleryl-CoA</a></span></div> <div id="annotation_85x198" style="position:absolute; left:85px; top:198px; font-size:15px; font-size:15; line-height:17px;"><span style="background-color:; color:inherit;"><a href="/wiki/%CE%92-Hydroxy_%CE%B2-methylbutyric_acid" title="Β-Hydroxy β-methylbutyric acid"><b>β-Hydroxy<br />β-methylbutyrate</b></a><br />(<b>HMB</b>)</span></div> <div id="annotation_2x205" style="position:absolute; left:2px; top:205px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Excretion" title="Excretion">Excreted</a><br />in urine<br />(10–40%)</span></div> <p><br /> </p> <div id="annotation_239x217" style="position:absolute; left:239px; top:217px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Beta-Hydroxy_beta-methylbutyryl-CoA" class="mw-redirect" title="Beta-Hydroxy beta-methylbutyryl-CoA">HMB-CoA</a></span></div> <div id="annotation_175x269" style="position:absolute; left:175px; top:269px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/HMG-CoA" title="HMG-CoA">β-Hydroxy β-methylglutaryl-CoA</a><br />(HMG-CoA)</span></div> <div id="annotation_445x208" style="position:absolute; left:445px; top:208px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Methylcrotonyl-CoA" title="Methylcrotonyl-CoA">β-Methylcrotonyl-CoA</a><br />(MC-CoA)</span></div> <div id="annotation_442x269" style="position:absolute; left:442px; top:269px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/3-Methylglutaconyl-CoA" title="3-Methylglutaconyl-CoA">β-Methylglutaconyl-CoA</a><br />(MG-CoA)</span></div> <div id="annotation_395x148" style="position:absolute; left:395px; top:148px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Carbon_dioxide" title="Carbon dioxide">CO<sub>2</sub></a></span></div> <div id="annotation_221x246" style="position:absolute; left:221px; top:246px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Carbon_dioxide" title="Carbon dioxide">CO<sub>2</sub></a></span></div> <div id="annotation_214x163" style="position:absolute; left:214px; top:163px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Oxygen" title="Oxygen">O<sub>2</sub></a></span></div> <div id="annotation_211x186" style="position:absolute; left:211px; top:186px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Carbon_dioxide" title="Carbon dioxide">CO<sub>2</sub></a></span></div> <div id="annotation_426x239" style="position:absolute; left:426px; top:239px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Properties_of_water" title="Properties of water">H<sub>2</sub>O</a></span></div> <div id="annotation_458x246" style="position:absolute; left:458px; top:246px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Carbon_dioxide" title="Carbon dioxide">CO<sub>2</sub></a></span></div> <div id="annotation_422x297" style="position:absolute; left:422px; top:297px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Properties_of_water" title="Properties of water">H<sub>2</sub>O</a></span></div> <div id="annotation_242x316" style="position:absolute; left:242px; top:316px; line-height:110%;"><span style="background-color:transparent; color:inherit;">(<a href="/wiki/Liver" title="Liver">liver</a>)<br /><a href="/wiki/HMG-CoA_lyase" class="mw-redirect" title="HMG-CoA lyase">HMG-CoA<br />lyase</a></span></div> <div id="annotation_314x208" style="position:absolute; left:314px; top:208px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Enoyl-CoA_hydratase" title="Enoyl-CoA hydratase">Enoyl-CoA hydratase</a></span></div> <div id="annotation_511x163" style="position:absolute; left:511px; top:163px; font-size:12px; font-size:12; line-height:14px; text-align:left;"><span style="background-color:transparent; color:inherit;"><span style="color:black;background:yellow"><b>Isovaleryl-CoA<br />dehydrogenase</b></span></span></div> <div id="annotation_511x238" style="position:absolute; left:511px; top:238px; font-size:12px; font-size:12; line-height:14px; text-align:left;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Methylcrotonyl-CoA_carboxylase" title="Methylcrotonyl-CoA carboxylase">MC-CoA<br />carboxylase</a></span></div> <div id="annotation_376x254" style="position:absolute; left:376px; top:254px; font-size:12px; font-size:12; line-height:14px; text-align:center;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Methylglutaconyl-CoA_hydratase" title="Methylglutaconyl-CoA hydratase">MG-CoA<br />hydratase</a></span></div> <div id="annotation_59x316" style="position:absolute; left:59px; top:316px; line-height:110%;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/HMG-CoA_reductase" title="HMG-CoA reductase">HMG-CoA<br />reductase</a></span></div> <div id="annotation_410x331" style="position:absolute; left:410px; top:331px; font-size:12px; font-size:12; line-height:14px; text-align:right;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/HMG-CoA_synthase" class="mw-redirect" title="HMG-CoA synthase">HMG-CoA <br />synthase</a></span></div> <div id="annotation_205x387" style="position:absolute; left:205px; top:387px; font-size:12px; font-size:12; line-height:14px; text-align:left;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/3-Hydroxybutyrate_dehydrogenase" title="3-Hydroxybutyrate dehydrogenase">β-Hydroxybutyrate<br />dehydrogenase</a></span></div> <div id="annotation_80x385" style="position:absolute; left:80px; top:385px; font-size:12px; font-size:12; line-height:14px; text-align:left;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/File:Mevalonate_pathway.svg" title="File:Mevalonate pathway.svg">Mevalonate<br />pathway</a></span></div> <div id="annotation_389x385" style="position:absolute; left:389px; top:385px; font-size:12px; font-size:12; line-height:14px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Thiolase" title="Thiolase">Thiolase</a></span></div> <div id="annotation_274x236" style="position:absolute; left:274px; top:236px; font-size:12px; font-size:12; line-height:14px; text-align:left;"><span style="background-color:transparent; color:inherit;">Unknown<br />enzyme</span></div> <div id="annotation_152x420" style="position:absolute; left:152px; top:420px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:; color:inherit;"><a href="/wiki/Beta-Hydroxybutyric_acid" class="mw-redirect" title="Beta-Hydroxybutyric acid">β-Hydroxybutyrate</a></span></div> <div id="annotation_432x395" style="position:absolute; left:432px; top:395px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Acetoacetyl-CoA" title="Acetoacetyl-CoA">Acetoacetyl-CoA</a></span></div> <div id="annotation_328x395" style="position:absolute; left:328px; top:395px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Acetyl-CoA" title="Acetyl-CoA">Acetyl-CoA</a></span></div> <div id="annotation_164x365" style="position:absolute; left:164px; top:365px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Acetoacetate" class="mw-redirect" title="Acetoacetate">Acetoacetate</a></span></div> <div id="annotation_44x365" style="position:absolute; left:44px; top:365px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Mevalonate" class="mw-redirect" title="Mevalonate">Mevalonate</a></span></div> <div id="annotation_43x420" style="position:absolute; left:43px; top:420px; font-size:13px; font-size:13; line-height:15px;"><span style="background-color:transparent; color:inherit;"><a href="/wiki/Cholesterol" title="Cholesterol">Cholesterol</a></span></div></div> </div> </div> <div class="thumbcaption" style="clear:left"><div style="float:left;margin-right:0.5em"><span typeof="mw:File"><span title="The image above contains clickable links"><img alt="The image above contains clickable links" src="//upload.wikimedia.org/wikipedia/commons/thumb/e/e6/Interactive_icon.svg/18px-Interactive_icon.svg.png" decoding="async" width="18" height="27" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/e/e6/Interactive_icon.svg/27px-Interactive_icon.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/e/e6/Interactive_icon.svg/36px-Interactive_icon.svg.png 2x" data-file-width="133" data-file-height="200" /></span></span></div>Human <a href="/wiki/Metabolic_pathway" title="Metabolic pathway">metabolic pathway</a> for <a href="/wiki/%CE%92-Hydroxy_%CE%B2-methylbutyric_acid" title="Β-Hydroxy β-methylbutyric acid">HMB</a> and <a href="/wiki/Isovaleryl-CoA" title="Isovaleryl-CoA">isovaleryl-CoA</a> relative to <a href="/wiki/Leucine" title="Leucine"><span class="nowrap"><span class="smallcaps"><span style="font-variant: small-caps; text-transform: lowercase;">L</span></span>-leucine</span></a>.<sup id="cite_ref-ISSN_position_stand_2013_1-0" class="reference"><a href="#cite_note-ISSN_position_stand_2013-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-HMB_athletic_performance-related_effects_2011_reviewb_2-0" class="reference"><a href="#cite_note-HMB_athletic_performance-related_effects_2011_reviewb-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Leucine_metabolism_3-0" class="reference"><a href="#cite_note-Leucine_metabolism-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> Of the two major pathways, <span class="nowrap"><span class="smallcaps"><span style="font-variant: small-caps; text-transform: lowercase;">L</span></span>-leucine</span> is mostly metabolized into isovaleryl-CoA, while only about 5% is metabolized into HMB.<sup id="cite_ref-ISSN_position_stand_2013_1-1" class="reference"><a href="#cite_note-ISSN_position_stand_2013-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-HMB_athletic_performance-related_effects_2011_reviewb_2-1" class="reference"><a href="#cite_note-HMB_athletic_performance-related_effects_2011_reviewb-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Leucine_metabolism_3-1" class="reference"><a href="#cite_note-Leucine_metabolism-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup></div> </div> </div> </td></tr></tbody></table> </div> <p><br /> </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=Isovaleryl-CoA_dehydrogenase&action=edit&section=3" 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-ISSN_position_stand_2013-1"><span class="mw-cite-backlink">^ <a href="#cite_ref-ISSN_position_stand_2013_1-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-ISSN_position_stand_2013_1-1"><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="CITEREFWilsonFitschenCampbellWilson2013" class="citation journal cs1">Wilson JM, Fitschen PJ, Campbell B, Wilson GJ, Zanchi N, Taylor L, Wilborn C, Kalman DS, Stout JR, Hoffman JR, Ziegenfuss TN, Lopez HL, Kreider RB, Smith-Ryan AE, Antonio J (February 2013). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3568064">"International Society of Sports Nutrition Position Stand: beta-hydroxy-beta-methylbutyrate (HMB)"</a>. <i>Journal of the International Society of Sports Nutrition</i>. <b>10</b> (1): 6. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1186%2F1550-2783-10-6">10.1186/1550-2783-10-6</a></span>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3568064">3568064</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/23374455">23374455</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+the+International+Society+of+Sports+Nutrition&rft.atitle=International+Society+of+Sports+Nutrition+Position+Stand%3A+beta-hydroxy-beta-methylbutyrate+%28HMB%29&rft.volume=10&rft.issue=1&rft.pages=6&rft.date=2013-02&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3568064%23id-name%3DPMC&rft_id=info%3Apmid%2F23374455&rft_id=info%3Adoi%2F10.1186%2F1550-2783-10-6&rft.aulast=Wilson&rft.aufirst=JM&rft.au=Fitschen%2C+PJ&rft.au=Campbell%2C+B&rft.au=Wilson%2C+GJ&rft.au=Zanchi%2C+N&rft.au=Taylor%2C+L&rft.au=Wilborn%2C+C&rft.au=Kalman%2C+DS&rft.au=Stout%2C+JR&rft.au=Hoffman%2C+JR&rft.au=Ziegenfuss%2C+TN&rft.au=Lopez%2C+HL&rft.au=Kreider%2C+RB&rft.au=Smith-Ryan%2C+AE&rft.au=Antonio%2C+J&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3568064&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIsovaleryl-CoA+dehydrogenase" class="Z3988"></span></span> </li> <li id="cite_note-HMB_athletic_performance-related_effects_2011_reviewb-2"><span class="mw-cite-backlink">^ <a href="#cite_ref-HMB_athletic_performance-related_effects_2011_reviewb_2-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-HMB_athletic_performance-related_effects_2011_reviewb_2-1"><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="CITEREFZanchiGerlinger-RomeroGuimarães-Ferreirade_Siqueira_Filho2011" class="citation journal cs1">Zanchi NE, Gerlinger-Romero F, Guimarães-Ferreira L, de Siqueira Filho MA, Felitti V, Lira FS, Seelaender M, Lancha AH (April 2011). <a rel="nofollow" class="external text" href="https://repositorio.unal.edu.co/handle/unal/77957">"HMB supplementation: clinical and athletic performance-related effects and mechanisms of action"</a>. <i>Amino Acids</i>. <b>40</b> (4): 1015–1025. <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%2Fs00726-010-0678-0">10.1007/s00726-010-0678-0</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/20607321">20607321</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:11120110">11120110</a>. <q>HMB is a metabolite of the amino acid leucine (Van Koverin and Nissen 1992), an essential amino acid. The first step in HMB metabolism is the reversible transamination of leucine to [α-KIC] that occurs mainly extrahepatically (Block and Buse 1990). Following this enzymatic reaction, [α-KIC] may follow one of two pathways. In the first, HMB is produced from [α-KIC] by the cytosolic enzyme KIC dioxygenase (Sabourin and Bieber 1983). The cytosolic dioxygenase has been characterized extensively and differs from the mitochondrial form in that the dioxygenase enzyme is a cytosolic enzyme, whereas the dehydrogenase enzyme is found exclusively in the mitochondrion (Sabourin and Bieber 1981, 1983). Importantly, this route of HMB formation is direct and completely dependent of liver KIC dioxygenase. Following this pathway, HMB in the cytosol is first converted to cytosolic β-hydroxy-β-methylglutaryl-CoA (HMG-CoA), which can then be directed for cholesterol synthesis (Rudney 1957) (Fig. 1). In fact, numerous biochemical studies have shown that HMB is a precursor of cholesterol (Zabin and Bloch 1951; Nissen et al. 2000).</q></cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Amino+Acids&rft.atitle=HMB+supplementation%3A+clinical+and+athletic+performance-related+effects+and+mechanisms+of+action&rft.volume=40&rft.issue=4&rft.pages=1015-1025&rft.date=2011-04&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A11120110%23id-name%3DS2CID&rft_id=info%3Apmid%2F20607321&rft_id=info%3Adoi%2F10.1007%2Fs00726-010-0678-0&rft.aulast=Zanchi&rft.aufirst=NE&rft.au=Gerlinger-Romero%2C+F&rft.au=Guimar%C3%A3es-Ferreira%2C+L&rft.au=de+Siqueira+Filho%2C+MA&rft.au=Felitti%2C+V&rft.au=Lira%2C+FS&rft.au=Seelaender%2C+M&rft.au=Lancha%2C+AH&rft_id=https%3A%2F%2Frepositorio.unal.edu.co%2Fhandle%2Funal%2F77957&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIsovaleryl-CoA+dehydrogenase" class="Z3988"></span></span> </li> <li id="cite_note-Leucine_metabolism-3"><span class="mw-cite-backlink">^ <a href="#cite_ref-Leucine_metabolism_3-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Leucine_metabolism_3-1"><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="CITEREFKohlmeier2015" class="citation book cs1">Kohlmeier M (May 2015). <a rel="nofollow" class="external text" href="https://books.google.com/books?id=aTQTAAAAQBAJ&q=beta-hydroxy%20beta-methylbutyrate%20HMB&pg=PA387">"Leucine"</a>. <a rel="nofollow" class="external text" href="https://books.google.com/books?id=aTQTAAAAQBAJ"><i>Nutrient Metabolism: Structures, Functions, and Genes</i></a> (2nd ed.). Academic Press. pp. 385–388. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/978-0-12-387784-0" title="Special:BookSources/978-0-12-387784-0"><bdi>978-0-12-387784-0</bdi></a><span class="reference-accessdate">. Retrieved <span class="nowrap">6 June</span> 2016</span>. <q>Energy fuel: Eventually, most Leu is broken down, providing about 6.0kcal/g. About 60% of ingested Leu is oxidized within a few hours ... Ketogenesis: A significant proportion (40% of an ingested dose) is converted into acetyl-CoA and thereby contributes to the synthesis of ketones, steroids, fatty acids, and other compounds</q></cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=bookitem&rft.atitle=Leucine&rft.btitle=Nutrient+Metabolism%3A+Structures%2C+Functions%2C+and+Genes&rft.pages=385-388&rft.edition=2nd&rft.pub=Academic+Press&rft.date=2015-05&rft.isbn=978-0-12-387784-0&rft.aulast=Kohlmeier&rft.aufirst=M&rft_id=https%3A%2F%2Fbooks.google.com%2Fbooks%3Fid%3DaTQTAAAAQBAJ%26q%3Dbeta-hydroxy%2520beta-methylbutyrate%2520HMB%26pg%3DPA387&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIsovaleryl-CoA+dehydrogenase" class="Z3988"></span><br /><a rel="nofollow" class="external text" href="https://books.google.com/books?id=aTQTAAAAQBAJ&pg=PA386#v=onepage&q&f=false">Figure 8.57: Metabolism of <span class="smallcaps"><span style="font-variant: small-caps; text-transform: lowercase;">L</span></span>-leucine</a></span> </li> </ol></div></div> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFBACHHAWATROBINSONCOON1956" class="citation journal cs1">BACHHAWAT BK, ROBINSON WG, COON MJ (1956). <a rel="nofollow" class="external text" href="https://doi.org/10.1016%2FS0021-9258%2818%2965714-X">"Enzymatic carboxylation of beta-hydroxyisovaleryl coenzyme A"</a>. <i>J. 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Chem</i>. <b>219</b> (2): 539–50. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1016%2FS0021-9258%2818%2965714-X">10.1016/S0021-9258(18)65714-X</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/13319276">13319276</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=J.+Biol.+Chem.&rft.atitle=Enzymatic+carboxylation+of+beta-hydroxyisovaleryl+coenzyme+A&rft.volume=219&rft.issue=2&rft.pages=539-50&rft.date=1956&rft_id=info%3Adoi%2F10.1016%2FS0021-9258%2818%2965714-X&rft_id=info%3Apmid%2F13319276&rft.aulast=BACHHAWAT&rft.aufirst=BK&rft.au=ROBINSON%2C+WG&rft.au=COON%2C+MJ&rft_id=https%3A%2F%2Fdoi.org%2F10.1016%252FS0021-9258%252818%252965714-X&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIsovaleryl-CoA+dehydrogenase" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFIkedaTanaka1983" class="citation journal cs1">Ikeda Y, Tanaka K (1983). <a rel="nofollow" class="external text" href="https://doi.org/10.1016%2FS0021-9258%2818%2933161-2">"Purification and characterization of isovaleryl coenzyme A dehydrogenase from rat liver mitochondria"</a>. <i>J. 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Chem</i>. <b>258</b> (2): 1077–85. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1016%2FS0021-9258%2818%2933161-2">10.1016/S0021-9258(18)33161-2</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/6401713">6401713</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=J.+Biol.+Chem.&rft.atitle=Purification+and+characterization+of+isovaleryl+coenzyme+A+dehydrogenase+from+rat+liver+mitochondria&rft.volume=258&rft.issue=2&rft.pages=1077-85&rft.date=1983&rft_id=info%3Adoi%2F10.1016%2FS0021-9258%2818%2933161-2&rft_id=info%3Apmid%2F6401713&rft.aulast=Ikeda&rft.aufirst=Y&rft.au=Tanaka%2C+K&rft_id=https%3A%2F%2Fdoi.org%2F10.1016%252FS0021-9258%252818%252933161-2&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIsovaleryl-CoA+dehydrogenase" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFTanakaBuddEfronIsselbacher1966" class="citation journal cs1">Tanaka K, Budd MA, Efron ML, Isselbacher KJ (1966). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC285701">"Isovaleric acidemia: a new genetic defect of leucine metabolism"</a>. <i>Proc. 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U.S.A</i>. <b>56</b> (1): 236–42. <a href="/wiki/Bibcode_(identifier)" class="mw-redirect" title="Bibcode (identifier)">Bibcode</a>:<a rel="nofollow" class="external text" href="https://ui.adsabs.harvard.edu/abs/1966PNAS...56..236T">1966PNAS...56..236T</a>. <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/10.1073%2Fpnas.56.1.236">10.1073/pnas.56.1.236</a></span>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC285701">285701</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/5229850">5229850</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Proc.+Natl.+Acad.+Sci.+U.S.A.&rft.atitle=Isovaleric+acidemia%3A+a+new+genetic+defect+of+leucine+metabolism&rft.volume=56&rft.issue=1&rft.pages=236-42&rft.date=1966&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC285701%23id-name%3DPMC&rft_id=info%3Apmid%2F5229850&rft_id=info%3Adoi%2F10.1073%2Fpnas.56.1.236&rft_id=info%3Abibcode%2F1966PNAS...56..236T&rft.aulast=Tanaka&rft.aufirst=K&rft.au=Budd%2C+MA&rft.au=Efron%2C+ML&rft.au=Isselbacher%2C+KJ&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC285701&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIsovaleryl-CoA+dehydrogenase" class="Z3988"></span></li></ul> <div class="navbox-styles"><style data-mw-deduplicate="TemplateStyles:r1129693374">.mw-parser-output .hlist dl,.mw-parser-output .hlist ol,.mw-parser-output .hlist ul{margin:0;padding:0}.mw-parser-output .hlist dd,.mw-parser-output .hlist dt,.mw-parser-output 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navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:CH-CH_oxidoreductases" title="Template:CH-CH oxidoreductases"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:CH-CH_oxidoreductases" title="Template talk:CH-CH oxidoreductases"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:CH-CH_oxidoreductases" title="Special:EditPage/Template:CH-CH oxidoreductases"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Oxidoreductases:_CH–CH_oxidoreductases_(EC_1.3)" style="font-size:114%;margin:0 4em"><a href="/wiki/Oxidoreductase" title="Oxidoreductase">Oxidoreductases</a>: <a href="/wiki/CH%E2%80%93CH_oxidoreductases" title="CH–CH oxidoreductases">CH–CH oxidoreductases</a> (<a href="/wiki/Enzyme_Commission_number" title="Enzyme Commission number">EC</a> 1.3)</div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/List_of_EC_numbers_(EC_1)#EC_1.3.1_With_NAD_or_NADP_as_acceptor" title="List of EC numbers (EC 1)">1.3.1</a>: <a href="/wiki/Nicotinamide_adenine_dinucleotide" title="Nicotinamide adenine dinucleotide">NAD</a>/<a href="/wiki/Nicotinamide_adenine_dinucleotide_phosphate" title="Nicotinamide adenine dinucleotide phosphate">NADP</a> acceptor</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/Enoyl-acyl_carrier_protein_reductase" title="Enoyl-acyl carrier protein reductase">Enoyl-acyl carrier protein reductase</a>/<a href="/wiki/Enoyl-acyl_carrier_protein_reductase" title="Enoyl-acyl carrier protein reductase">Enoyl ACP reductase</a></li> <li><a href="/wiki/7-Dehydrocholesterol_reductase" title="7-Dehydrocholesterol reductase">7-Dehydrocholesterol reductase</a></li> <li><a href="/wiki/Biliverdin_reductase" title="Biliverdin reductase">Biliverdin reductase</a></li> <li><a href="/wiki/2,4_Dienoyl-CoA_reductase" title="2,4 Dienoyl-CoA reductase">2,4 Dienoyl-CoA reductase</a></li> <li><a href="/wiki/5,6-dihydroxy-3-methyl-2-oxo-1,2,5,6-tetrahydroquinoline_dehydrogenase" title="5,6-dihydroxy-3-methyl-2-oxo-1,2,5,6-tetrahydroquinoline dehydrogenase">Dihydroxymethyloxo-tetrahydroquinoline dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/List_of_EC_numbers_(EC_1)#EC_1.3.3_With_oxygen_as_acceptor" title="List of EC numbers (EC 1)">1.3.3</a>: <a href="/wiki/Oxygen" title="Oxygen">Oxygen</a> acceptor</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/Dihydroorotate_dehydrogenase" title="Dihydroorotate dehydrogenase">Dihydroorotate dehydrogenase</a></li> <li><a href="/wiki/Coproporphyrinogen_III_oxidase" title="Coproporphyrinogen III oxidase">Coproporphyrinogen III oxidase</a></li> <li><a href="/wiki/Protoporphyrinogen_oxidase" title="Protoporphyrinogen oxidase">Protoporphyrinogen oxidase</a></li> <li><a href="/wiki/Bilirubin_oxidase" title="Bilirubin oxidase">Bilirubin oxidase</a></li> <li><a href="/wiki/Acyl-CoA_oxidase" title="Acyl-CoA oxidase">Acyl-CoA oxidase</a></li> <li><a href="/wiki/Dihydrouracil_oxidase" title="Dihydrouracil oxidase">Dihydrouracil oxidase</a></li> <li><a href="/wiki/Tetrahydroberberine_oxidase" title="Tetrahydroberberine oxidase">Tetrahydroberberine oxidase</a></li> <li><a href="/wiki/Secologanin_synthase" title="Secologanin synthase">Secologanin synthase</a></li> <li><a href="/wiki/Tryptophan_alpha,beta-oxidase" title="Tryptophan alpha,beta-oxidase">Tryptophan alpha,beta-oxidase</a></li> <li><a href="/wiki/Pyrroloquinoline-quinone_synthase" title="Pyrroloquinoline-quinone synthase">Pyrroloquinoline-quinone synthase</a></li> <li><a href="/wiki/L-galactonolactone_oxidase" title="L-galactonolactone oxidase">L-galactonolactone oxidase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/List_of_EC_numbers_(EC_1)#EC_1.3.5_With_a_quinone_or_related_compound_as_acceptor" title="List of EC numbers (EC 1)">1.3.5</a>: <a href="/wiki/Quinone" title="Quinone">Quinone</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Succinate_dehydrogenase" title="Succinate dehydrogenase">Succinate dehydrogenase</a> <ul><li><a href="/wiki/SDHA" title="SDHA">SDHA</a></li> <li><a href="/wiki/SDHB" title="SDHB">SDHB</a></li> <li><a href="/wiki/Succinate_dehydrogenase_complex_subunit_C" title="Succinate dehydrogenase complex subunit C">SDHC</a></li> <li><a href="/wiki/SDHD" title="SDHD">SDHD</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_1)#EC_1.1.99_With_other_acceptors" title="List of EC numbers (EC 1)">1.3.99</a>: Other acceptors</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/Fumarate_reductase" title="Fumarate reductase">Fumarate reductase</a></li> <li><a href="/wiki/Butyryl-CoA_dehydrogenase" class="mw-redirect" title="Butyryl-CoA dehydrogenase">Butyryl-CoA dehydrogenase</a></li> <li><a href="/wiki/Acyl_CoA_dehydrogenase" class="mw-redirect" title="Acyl CoA dehydrogenase">Acyl CoA dehydrogenase</a> <ul><li><a href="/wiki/ACADSB" title="ACADSB">ACADSB</a></li> <li><a href="/wiki/ACADS" title="ACADS">ACADS</a></li></ul></li> <li><a href="/wiki/5-alpha_reductase" class="mw-redirect" title="5-alpha reductase">5α-reductase</a> <ul><li><a href="/wiki/SRD5A1" title="SRD5A1">SRD5A1</a></li> <li><a href="/wiki/SRD5A2" title="SRD5A2">SRD5A2</a></li> <li><a href="/wiki/SRD5A3" title="SRD5A3">SRD5A3</a></li></ul></li> <li><a href="/wiki/Glutaryl-CoA_dehydrogenase" title="Glutaryl-CoA dehydrogenase">Glutaryl-CoA dehydrogenase</a></li> <li><a class="mw-selflink selflink">Isovaleryl coenzyme A dehydrogenase</a></li> <li><a href="/wiki/3-oxo-5beta-steroid_4-dehydrogenase" title="3-oxo-5beta-steroid 4-dehydrogenase">3-oxo-5beta-steroid 4-dehydrogenase</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> 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