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mw-first-heading"><span class="mw-page-title-main">Glutathione synthetase</span></h1> <div id="p-lang-btn" class="vector-dropdown mw-portlet mw-portlet-lang" > <input type="checkbox" id="p-lang-btn-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-p-lang-btn" class="vector-dropdown-checkbox mw-interlanguage-selector" aria-label="Go to an article in another language. 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href="https://de.wikipedia.org/wiki/Glutathionsynthase" title="Glutathionsynthase – German" lang="de" hreflang="de" data-title="Glutathionsynthase" data-language-autonym="Deutsch" data-language-local-name="German" class="interlanguage-link-target"><span>Deutsch</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/Glutathion_synthase" title="Glutathion synthase – French" lang="fr" hreflang="fr" data-title="Glutathion synthase" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-hy mw-list-item"><a href="https://hy.wikipedia.org/wiki/%D4%B3%D5%AC%D5%B8%D6%82%D5%BF%D5%A1%D5%A9%D5%AB%D5%B8%D5%B6_%D5%BD%D5%AB%D5%B6%D5%A9%D5%A5%D5%BF%D5%A1%D5%A6" title="Գլուտաթիոն սինթետազ – Armenian" lang="hy" hreflang="hy" data-title="Գլուտաթիոն սինթետազ" data-language-autonym="Հայերեն" data-language-local-name="Armenian" class="interlanguage-link-target"><span>Հայերեն</span></a></li><li class="interlanguage-link interwiki-ja mw-list-item"><a href="https://ja.wikipedia.org/wiki/%E3%82%B0%E3%83%AB%E3%82%BF%E3%83%81%E3%82%AA%E3%83%B3%E5%90%88%E6%88%90%E9%85%B5%E7%B4%A0" title="グルタチオン合成酵素 – Japanese" lang="ja" hreflang="ja" data-title="グルタチオン合成酵素" data-language-autonym="日本語" data-language-local-name="Japanese" class="interlanguage-link-target"><span>日本語</span></a></li><li class="interlanguage-link interwiki-pl mw-list-item"><a href="https://pl.wikipedia.org/wiki/Syntetaza_glutationowa" title="Syntetaza glutationowa – Polish" lang="pl" hreflang="pl" data-title="Syntetaza glutationowa" data-language-autonym="Polski" data-language-local-name="Polish" class="interlanguage-link-target"><span>Polski</span></a></li><li class="interlanguage-link interwiki-sr mw-list-item"><a href="https://sr.wikipedia.org/wiki/Glutationska_sintaza" title="Glutationska sintaza – Serbian" lang="sr" hreflang="sr" data-title="Glutationska sintaza" data-language-autonym="Српски / srpski" data-language-local-name="Serbian" class="interlanguage-link-target"><span>Српски / srpski</span></a></li><li class="interlanguage-link interwiki-sh mw-list-item"><a href="https://sh.wikipedia.org/wiki/Glutationska_sintaza" title="Glutationska sintaza – Serbo-Croatian" lang="sh" hreflang="sh" data-title="Glutationska sintaza" data-language-autonym="Srpskohrvatski / српскохрватски" data-language-local-name="Serbo-Croatian" class="interlanguage-link-target"><span>Srpskohrvatski / српскохрватски</span></a></li> </ul> <div class="after-portlet after-portlet-lang"><span class="wb-langlinks-edit wb-langlinks-link"><a href="https://www.wikidata.org/wiki/Special:EntityPage/Q106950316#sitelinks-wikipedia" title="Edit interlanguage links" class="wbc-editpage">Edit links</a></span></div> </div> </div> </div> </header> <div class="vector-page-toolbar"> <div class="vector-page-toolbar-container"> <div id="left-navigation"> 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class="infobox"><tbody><tr><th colspan="2" class="infobox-above">Glutathione synthetase</th></tr><tr><td colspan="2" class="infobox-image"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:Glutathione_synthetase_1M0W.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/f4/Glutathione_synthetase_1M0W.png/220px-Glutathione_synthetase_1M0W.png" decoding="async" width="220" height="128" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/f4/Glutathione_synthetase_1M0W.png/330px-Glutathione_synthetase_1M0W.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/f4/Glutathione_synthetase_1M0W.png/440px-Glutathione_synthetase_1M0W.png 2x" data-file-width="635" data-file-height="370" /></a></span><div class="infobox-caption">Structure of glutathione synthetase in <a href="/wiki/Yeast" title="Yeast">yeast</a>. Generated from 1M0W.<sup id="cite_ref-Gogos_2002_1-0" class="reference"><a href="#cite_note-Gogos_2002-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup></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">GSS</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/National_Center_for_Biotechnology_Information#Gene" title="National Center for Biotechnology Information">NCBI gene</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/gene?cmd=retrieve&dopt=default&list_uids=2937&rn=1">2937</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:4624">4624</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/601002">601002</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_000178">NM_000178</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/P48637">P48637</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+6.3.2.3">6.3.2.3</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_20_(human)" class="mw-redirect" title="Chromosome 20 (human)">Chr. 20</a> <i><a rel="nofollow" class="external text" href="https://omim.org/search/?index=geneMap&search=20q11.2">q11.2</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/P48637">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/P48637">InterPro</a></td></tr></tbody></table></td></tr></tbody></table> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1257001546"><table class="infobox"><tbody><tr><th colspan="2" class="infobox-above">Eukaryotic glutathione synthase</th></tr><tr><td colspan="2" class="infobox-image"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:PDB_2hgs_EBI.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/7/78/PDB_2hgs_EBI.jpg/220px-PDB_2hgs_EBI.jpg" decoding="async" width="220" height="165" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/7/78/PDB_2hgs_EBI.jpg/330px-PDB_2hgs_EBI.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/7/78/PDB_2hgs_EBI.jpg/440px-PDB_2hgs_EBI.jpg 2x" data-file-width="800" data-file-height="600" /></a></span><div class="infobox-caption">Human glutathione synthetase</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">GSH_synthase</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Pfam" title="Pfam">Pfam</a></th><td class="infobox-data pfam" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/pfam/PF03199">PF03199</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Pfam" title="Pfam">Pfam</a> clan</th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/set/pfam/CL0483/">CL0483</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/InterPro" title="InterPro">InterPro</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/IPR004887">IPR004887</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Structural_Classification_of_Proteins" class="mw-redirect" title="Structural Classification of Proteins">SCOP2</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="http://scop2.mrc-lmb.cam.ac.uk/search?t=txt;q=2hgs">2hgs</a> / <a rel="nofollow" class="external text" href="https://scop.berkeley.edu/pdb/code=2hgs">SCOPe</a> / <a rel="nofollow" class="external text" href="http://supfam.org/SUPERFAMILY/cgi-bin/search.cgi?search_field=2hgs">SUPFAM</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">Available protein structures:</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/Pfam" title="Pfam">Pfam</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="http://pfam.xfam.org/family/PF03199?tab=pdbBlock">structures</a> / <a rel="nofollow" class="external text" href="http://prodata.swmed.edu/ecod/complete/search?kw=PF03199">ECOD</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/Protein_Data_Bank" title="Protein Data Bank">PDB</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.rcsb.org/search?q=rcsb_polymer_entity_annotation.annotation_id:PF03199%20AND%20rcsb_polymer_entity_annotation.type:Pfam">RCSB PDB</a>; <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/pdbe/entry/search/index?pfam_accession:PF03199">PDBe</a>; <a rel="nofollow" class="external text" href="https://pdbj.org/search/pdb?other_db_select=PFam&other_db_field=PF03199">PDBj</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/PDBsum" title="PDBsum">PDBsum</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.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPfamStr.pl?pfam_id=PF03199">structure summary</a></td></tr></tbody></table></td></tr></tbody></table> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1257001546"><table class="infobox"><tbody><tr><th colspan="2" class="infobox-above">glutathione synthase</th></tr><tr><td colspan="2" class="infobox-image"><span typeof="mw:File"><a href="/wiki/File:2hgs.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/e/e8/2hgs.jpg/270px-2hgs.jpg" decoding="async" width="270" height="215" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/e/e8/2hgs.jpg/405px-2hgs.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/e/e8/2hgs.jpg/540px-2hgs.jpg 2x" data-file-width="1000" data-file-height="797" /></a></span><div class="infobox-caption">glutathione synthetase dimer, 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=6.3.2.3">6.3.2.3</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=9023-62-5&title=">9023-62-5 </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=6.3.2.3">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=6.3.2.3">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/6.3.2.3">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+6.3.2.3">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=6.3.2.3">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=6.3.2.3">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:6.3.2.3">RCSB PDB</a> <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/pdbe/entry/search/index?ec_number:6.3.2.3">PDBe</a> <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/enzymes/GetPage.pl?ec_number=6.3.2.3">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:0004363">AmiGO </a> / <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/QuickGO/term/GO:0004363">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=6.3.2.3%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=6.3.2.3%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=6.3.2.3%5BEC/RN%20Number%5D">proteins</a></td></tr></tbody></table></td></tr></tbody></table> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1257001546"><table class="infobox"><tbody><tr><th colspan="2" class="infobox-above">Eukaryotic glutathione synthase, ATP binding domain</th></tr><tr><td colspan="2" class="infobox-image"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:PDB_2hgs_EBI.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/7/78/PDB_2hgs_EBI.jpg/220px-PDB_2hgs_EBI.jpg" decoding="async" width="220" height="165" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/7/78/PDB_2hgs_EBI.jpg/330px-PDB_2hgs_EBI.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/7/78/PDB_2hgs_EBI.jpg/440px-PDB_2hgs_EBI.jpg 2x" data-file-width="800" data-file-height="600" /></a></span><div class="infobox-caption">Human glutathione synthetase</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">GSH_synth_ATP</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Pfam" title="Pfam">Pfam</a></th><td class="infobox-data pfam" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/pfam/PF03917">PF03917</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/InterPro" title="InterPro">InterPro</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/IPR005615">IPR005615</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Structural_Classification_of_Proteins" class="mw-redirect" title="Structural Classification of Proteins">SCOP2</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="http://scop2.mrc-lmb.cam.ac.uk/search?t=txt;q=1m0t">1m0t</a> / <a rel="nofollow" class="external text" href="https://scop.berkeley.edu/pdb/code=1m0t">SCOPe</a> / <a rel="nofollow" class="external text" href="http://supfam.org/SUPERFAMILY/cgi-bin/search.cgi?search_field=1m0t">SUPFAM</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">Available protein structures:</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/Pfam" title="Pfam">Pfam</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="http://pfam.xfam.org/family/PF03917?tab=pdbBlock">structures</a> / <a rel="nofollow" class="external text" href="http://prodata.swmed.edu/ecod/complete/search?kw=PF03917">ECOD</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/Protein_Data_Bank" title="Protein Data Bank">PDB</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.rcsb.org/search?q=rcsb_polymer_entity_annotation.annotation_id:PF03917%20AND%20rcsb_polymer_entity_annotation.type:Pfam">RCSB PDB</a>; <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/pdbe/entry/search/index?pfam_accession:PF03917">PDBe</a>; <a rel="nofollow" class="external text" href="https://pdbj.org/search/pdb?other_db_select=PFam&other_db_field=PF03917">PDBj</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/PDBsum" title="PDBsum">PDBsum</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.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPfamStr.pl?pfam_id=PF03917">structure summary</a></td></tr></tbody></table></td></tr></tbody></table> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1257001546"><table class="infobox"><tbody><tr><th colspan="2" class="infobox-above">Prokaryotic glutathione synthetase, N-terminal domain</th></tr><tr><td colspan="2" class="infobox-image"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:PDB_1gsh_EBI.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/fd/PDB_1gsh_EBI.jpg/220px-PDB_1gsh_EBI.jpg" decoding="async" width="220" height="165" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/fd/PDB_1gsh_EBI.jpg/330px-PDB_1gsh_EBI.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/fd/PDB_1gsh_EBI.jpg/440px-PDB_1gsh_EBI.jpg 2x" data-file-width="800" data-file-height="600" /></a></span><div class="infobox-caption">Structure of escherichia coli glutathione synthetase at ph 7.5</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">GSH-S_N</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Pfam" title="Pfam">Pfam</a></th><td class="infobox-data pfam" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/pfam/PF02951">PF02951</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/InterPro" title="InterPro">InterPro</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/IPR004215">IPR004215</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Structural_Classification_of_Proteins" class="mw-redirect" title="Structural Classification of Proteins">SCOP2</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="http://scop2.mrc-lmb.cam.ac.uk/search?t=txt;q=1glv">1glv</a> / <a rel="nofollow" class="external text" href="https://scop.berkeley.edu/pdb/code=1glv">SCOPe</a> / <a rel="nofollow" class="external text" href="http://supfam.org/SUPERFAMILY/cgi-bin/search.cgi?search_field=1glv">SUPFAM</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">Available protein structures:</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/Pfam" title="Pfam">Pfam</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="http://pfam.xfam.org/family/PF02951?tab=pdbBlock">structures</a> / <a rel="nofollow" class="external text" href="http://prodata.swmed.edu/ecod/complete/search?kw=PF02951">ECOD</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/Protein_Data_Bank" title="Protein Data Bank">PDB</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.rcsb.org/search?q=rcsb_polymer_entity_annotation.annotation_id:PF02951%20AND%20rcsb_polymer_entity_annotation.type:Pfam">RCSB PDB</a>; <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/pdbe/entry/search/index?pfam_accession:PF02951">PDBe</a>; <a rel="nofollow" class="external text" href="https://pdbj.org/search/pdb?other_db_select=PFam&other_db_field=PF02951">PDBj</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/PDBsum" title="PDBsum">PDBsum</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.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPfamStr.pl?pfam_id=PF02951">structure summary</a></td></tr></tbody></table></td></tr></tbody></table> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1257001546"><table class="infobox"><tbody><tr><th colspan="2" class="infobox-above">Prokaryotic glutathione synthetase, ATP-grasp domain</th></tr><tr><td colspan="2" class="infobox-image"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:PDB_1gsh_EBI.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/fd/PDB_1gsh_EBI.jpg/220px-PDB_1gsh_EBI.jpg" decoding="async" width="220" height="165" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/fd/PDB_1gsh_EBI.jpg/330px-PDB_1gsh_EBI.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/fd/PDB_1gsh_EBI.jpg/440px-PDB_1gsh_EBI.jpg 2x" data-file-width="800" data-file-height="600" /></a></span><div class="infobox-caption">Structure of escherichia coli glutathione synthetase at ph 7.5</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">GSH-S_ATP</td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Pfam" title="Pfam">Pfam</a></th><td class="infobox-data pfam" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/pfam/PF02955">PF02955</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Pfam" title="Pfam">Pfam</a> clan</th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/set/pfam/CL0179/">CL0179</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/InterPro" title="InterPro">InterPro</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/interpro/entry/IPR004218">IPR004218</a></td></tr><tr><th scope="row" class="infobox-label" style="background-color: #e7dcc3"><a href="/wiki/Structural_Classification_of_Proteins" class="mw-redirect" title="Structural Classification of Proteins">SCOP2</a></th><td class="infobox-data" style="background-color: #eee"><a rel="nofollow" class="external text" href="http://scop2.mrc-lmb.cam.ac.uk/search?t=txt;q=1glv">1glv</a> / <a rel="nofollow" class="external text" href="https://scop.berkeley.edu/pdb/code=1glv">SCOPe</a> / <a rel="nofollow" class="external text" href="http://supfam.org/SUPERFAMILY/cgi-bin/search.cgi?search_field=1glv">SUPFAM</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">Available protein structures:</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/Pfam" title="Pfam">Pfam</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="http://pfam.xfam.org/family/PF02955?tab=pdbBlock">structures</a> / <a rel="nofollow" class="external text" href="http://prodata.swmed.edu/ecod/complete/search?kw=PF02955">ECOD</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/Protein_Data_Bank" title="Protein Data Bank">PDB</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.rcsb.org/search?q=rcsb_polymer_entity_annotation.annotation_id:PF02955%20AND%20rcsb_polymer_entity_annotation.type:Pfam">RCSB PDB</a>; <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/pdbe/entry/search/index?pfam_accession:PF02955">PDBe</a>; <a rel="nofollow" class="external text" href="https://pdbj.org/search/pdb?other_db_select=PFam&other_db_field=PF02955">PDBj</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/PDBsum" title="PDBsum">PDBsum</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.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPfamStr.pl?pfam_id=PF02955">structure summary</a></td></tr></tbody></table></td></tr></tbody></table> <p><b>Glutathione synthetase</b> (<b>GSS</b>) (<a href="/wiki/Enzyme_Commission_number" title="Enzyme Commission number">EC</a> <a rel="nofollow" class="external text" href="https://www.enzyme-database.org/query.php?ec=6.3.2.3">6.3.2.3</a>) is the second enzyme in the <a href="/wiki/Glutathione" title="Glutathione">glutathione</a> (GSH) biosynthesis pathway. It catalyses the condensation of <a href="/wiki/Gamma-glutamylcysteine" class="mw-redirect" title="Gamma-glutamylcysteine">gamma-glutamylcysteine</a> and <a href="/wiki/Glycine" title="Glycine">glycine</a>, to form glutathione.<sup id="cite_ref-2" class="reference"><a href="#cite_note-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Glutathione synthetase is also a potent antioxidant. It is found in many species including bacteria, yeast, mammals, and plants.<sup id="cite_ref-Li_2003_3-0" class="reference"><a href="#cite_note-Li_2003-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>In humans, defects in GSS are inherited in an <a href="/wiki/Autosomal_recessive" class="mw-redirect" title="Autosomal recessive">autosomal recessive</a> way and are the cause of severe <a href="/wiki/Metabolic_acidosis" title="Metabolic acidosis">metabolic acidosis</a>, <a href="/wiki/5-oxoprolinuria" class="mw-redirect" title="5-oxoprolinuria">5-oxoprolinuria</a>, increased rate of <a href="/wiki/Haemolysis" class="mw-redirect" title="Haemolysis">haemolysis</a>, and defective function of the <a href="/wiki/Central_nervous_system" title="Central nervous system">central nervous system</a>.<sup id="cite_ref-Njålsson_2005_4-0" class="reference"><a href="#cite_note-Njålsson_2005-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Deficiencies in GSS can cause a spectrum of deleterious symptoms in plants and human beings alike.<sup id="cite_ref-O'Neill_2005_5-0" class="reference"><a href="#cite_note-O'Neill_2005-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p><p>In <a href="/wiki/Eukaryotes" class="mw-redirect" title="Eukaryotes">eukaryotes</a>, this is a <a href="/wiki/Protein_dimer" title="Protein dimer">homodimeric</a> enzyme. The substrate-binding domain has a three-layer <a href="/wiki/Alpha_helix" title="Alpha helix">alpha</a>/<a href="/wiki/Beta_sheet" title="Beta sheet">beta</a>/alpha <a href="/wiki/Secondary_structure" class="mw-redirect" title="Secondary structure">structure</a>.<sup id="cite_ref-Polekhina_1999_6-0" class="reference"><a href="#cite_note-Polekhina_1999-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> This enzyme utilizes and stabilizes an acylphosphate intermediate to later perform a favorable <a href="/wiki/Nucleophilic_substitution" title="Nucleophilic substitution">nucleophilic attack</a> of <a href="/wiki/Glycine" title="Glycine">glycine</a>.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (November 2024)">citation needed</span></a></i>]</sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Structure">Structure</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Glutathione_synthetase&action=edit&section=1" title="Edit section: Structure"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Human and yeast glutathione synthetases are <a href="/wiki/Protein_dimer" title="Protein dimer">homodimers</a>, meaning they are composed of two identical <a href="/wiki/Protein_subunit" title="Protein subunit">subunits</a> of itself <a href="/wiki/Non-covalent_interactions" class="mw-redirect" title="Non-covalent interactions">non-covalently</a> bound to each other. On the other hand, <i>E. coli</i> glutathione synthetase is a <a href="/wiki/Tetramer" title="Tetramer">homotetramer</a>.<sup id="cite_ref-Li_2003_3-1" class="reference"><a href="#cite_note-Li_2003-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> Nevertheless, they are part of the <a href="/wiki/ATP-grasp" title="ATP-grasp">ATP-grasp</a> <a href="/wiki/Protein_superfamily" title="Protein superfamily">superfamily</a>, which consists of 21 enzymes that contain an ATP-grasp fold.<sup id="cite_ref-Banerjee_2007_7-0" class="reference"><a href="#cite_note-Banerjee_2007-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> Each subunit interacts with each other through <a href="/wiki/Alpha_helix" title="Alpha helix">alpha helix</a> and <a href="/wiki/Beta_sheet" title="Beta sheet">beta sheet</a> <a href="/wiki/Hydrogen_bond" title="Hydrogen bond">hydrogen bonding</a> interactions and contains two domains. One domain facilitates the ATP-grasp mechanism<sup id="cite_ref-Fawaz_2011_8-0" class="reference"><a href="#cite_note-Fawaz_2011-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> and the other is the catalytic <a href="/wiki/Active_site" title="Active site">active site</a> for <a href="/wiki/Gamma-glutamylcysteine" class="mw-redirect" title="Gamma-glutamylcysteine">γ-glutamylcysteine</a>. The <a href="/w/index.php?title=ATP-grasp_fold&action=edit&redlink=1" class="new" title="ATP-grasp fold (page does not exist)">ATP-grasp fold</a> is conserved within the ATP-grasp superfamily and is characterized by two alpha helices and beta sheets that hold onto the ATP molecule between them.<sup id="cite_ref-Fyfe_2010_9-0" class="reference"><a href="#cite_note-Fyfe_2010-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> The domain containing the active site exhibits interesting properties of specificity. In contrast to γ-glutamylcysteine synthetase, glutathione synthetase accepts a large variety of glutamyl-modified analogs of γ-glutamylcysteine, but is much more specific for cysteine-modified analogs of γ-glutamylcysteine.<sup id="cite_ref-Galperin_1997_10-0" class="reference"><a href="#cite_note-Galperin_1997-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> Crystalline structures have shown glutathione synthetase bound to GSH, ADP, two <a href="/wiki/Magnesium" title="Magnesium">magnesium</a> ions, and a sulfate ion.<sup id="cite_ref-Meister_1978_11-0" class="reference"><a href="#cite_note-Meister_1978-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> Two magnesium ions function to stabilize the acylphosphate intermediate, facilitate binding of ATP, and activate removal of phosphate group from ATP. Sulfate ion serves as a replacement for inorganic phosphate once the acylphosphate intermediate is formed inside the active site.<sup id="cite_ref-Polekhina_1999_6-1" class="reference"><a href="#cite_note-Polekhina_1999-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <figure class="mw-default-size mw-halign-center" typeof="mw:File/Thumb"><a href="/wiki/File:Key_residues_that_interact_with_ADP_within_the_active_site.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/d/d5/Key_residues_that_interact_with_ADP_within_the_active_site.png/310px-Key_residues_that_interact_with_ADP_within_the_active_site.png" decoding="async" width="310" height="386" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/d/d5/Key_residues_that_interact_with_ADP_within_the_active_site.png/465px-Key_residues_that_interact_with_ADP_within_the_active_site.png 1.5x, //upload.wikimedia.org/wikipedia/commons/d/d5/Key_residues_that_interact_with_ADP_within_the_active_site.png 2x" data-file-width="574" data-file-height="714" /></a><figcaption>Key residues that interact with ATP near the active site. Magnesium ions are shown in black. Generated from 2HGS.<sup id="cite_ref-Polekhina_1999_6-2" class="reference"><a href="#cite_note-Polekhina_1999-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup></figcaption></figure> <p>As of late 2007, 7 <a href="/wiki/Tertiary_structure" class="mw-redirect" title="Tertiary structure">structures</a> have been solved for this class of enzymes, with <a href="/wiki/Protein_Data_Bank" title="Protein Data Bank">PDB</a> accession codes <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=1GLV">1GLV</a>, <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=1GSA">1GSA</a>, <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=1GSH">1GSH</a>, <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=1M0T">1M0T</a>, <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=1M0W">1M0W</a>, <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=2GLT">2GLT</a>, and <a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl?pdbcode=2HGS">2HGS</a>. </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=Glutathione_synthetase&action=edit&section=2" title="Edit section: Mechanism"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Glutathione synthase <a href="/wiki/Catalysis" title="Catalysis">catalyzes</a> the <a href="/wiki/Chemical_reaction" title="Chemical reaction">chemical reaction</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (November 2024)">citation needed</span></a></i>]</sup> </p> <dl><dd>ATP + gamma-L-glutamyl-L-cysteine + glycine <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> ADP + phosphate + glutathione</dd></dl> <p>The 3 <a href="/wiki/Substrate_(biochemistry)" class="mw-redirect" title="Substrate (biochemistry)">substrates</a> of this enzyme are <a href="/wiki/Adenosine_triphosphate" title="Adenosine triphosphate">ATP</a>, <a href="/wiki/Gamma-L-glutamyl-L-cysteine" class="mw-redirect" title="Gamma-L-glutamyl-L-cysteine">gamma-L-glutamyl-L-cysteine</a>, and <a href="/wiki/Glycine" title="Glycine">glycine</a>, whereas its 3 <a href="/wiki/Product_(chemistry)" title="Product (chemistry)">products</a> are <a href="/wiki/Adenosine_diphosphate" title="Adenosine diphosphate">ADP</a>, <a href="/wiki/Phosphate" title="Phosphate">phosphate</a>, and <a href="/wiki/Glutathione" title="Glutathione">glutathione</a>. </p><p>This enzyme belongs to the family of <a href="/wiki/Ligase" title="Ligase">ligases</a>, specifically those forming carbon-nitrogen bonds as acid-D-amino-acid ligases (peptide synthases). The <a href="/wiki/List_of_enzymes" title="List of enzymes">systematic name</a> of this enzyme class is <b>gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)</b>. Other names in common use include <b>glutathione synthetase</b>, and <b>GSH synthetase</b>. This enzyme participates in <a href="/wiki/Glutamate_metabolism" class="mw-redirect" title="Glutamate metabolism">glutamate metabolism</a> and <a href="/wiki/Glutathione_metabolism" class="mw-redirect" title="Glutathione metabolism">glutathione metabolism</a>. At least one compound, <a href="/wiki/Phosphinate" title="Phosphinate">Phosphinate</a> is known to <a href="/wiki/Enzyme_inhibitor" title="Enzyme inhibitor">inhibit this enzyme</a>.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (November 2024)">citation needed</span></a></i>]</sup> </p><p>The biosynthetic mechanisms for <a href="/wiki/Ligase" title="Ligase">synthetases</a> use energy from <a href="/wiki/Nucleoside_triphosphates" class="mw-redirect" title="Nucleoside triphosphates">nucleoside triphosphates</a>, whereas <a href="/wiki/Synthases" class="mw-redirect" title="Synthases">synthases</a> do not.<sup id="cite_ref-Hara_1996_12-0" class="reference"><a href="#cite_note-Hara_1996-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Glutathione synthetase stays true to this rule, in that it uses the energy generated by ATP. Initially, the <a href="/wiki/Carboxylate" title="Carboxylate">carboxylate</a> group on γ-glutamylcysteine is converted into an <a href="/wiki/Acyl" class="mw-redirect" title="Acyl">acyl</a> phosphate by the transfer of an inorganic phosphate group of ATP to generate an acyl phosphate intermediate. Then the amino group of glycine participates in a nucleophilic attack, displacing the phosphate group and forming GSH.<sup id="cite_ref-urlsynthase_and_ligase_13-0" class="reference"><a href="#cite_note-urlsynthase_and_ligase-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> After the final GSH product is made, it can be used by <a href="/wiki/Glutathione_peroxidase" title="Glutathione peroxidase">glutathione peroxidase</a> to neutralize <a href="/wiki/Reactive_oxygen_species" title="Reactive oxygen species">reactive oxygen species</a> (ROS) such as H<sub>2</sub>O<sub>2</sub> or <a href="/wiki/Glutathione_S-transferase" title="Glutathione S-transferase">Glutathione S-transferases</a> in the detoxification of <a href="/wiki/Xenobiotics" class="mw-redirect" title="Xenobiotics">xenobiotics</a>.<sup id="cite_ref-Banerjee_2007_7-1" class="reference"><a href="#cite_note-Banerjee_2007-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <figure class="mw-default-size mw-halign-center" typeof="mw:File/Thumb"><a href="/wiki/File:Overall_synthesis_rxn_of_GSH.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/d/db/Overall_synthesis_rxn_of_GSH.jpg/530px-Overall_synthesis_rxn_of_GSH.jpg" decoding="async" width="530" height="583" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/d/db/Overall_synthesis_rxn_of_GSH.jpg/795px-Overall_synthesis_rxn_of_GSH.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/d/db/Overall_synthesis_rxn_of_GSH.jpg 2x" data-file-width="866" data-file-height="952" /></a><figcaption>Reaction mechanism for GSH biosynthesis.<sup id="cite_ref-Herrera_2007_14-0" class="reference"><a href="#cite_note-Herrera_2007-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Glutamate" class="mw-redirect" title="Glutamate">Glutamate</a> and <a href="/wiki/Cysteine" title="Cysteine">cysteine</a> side chains are shown in red and green, respectively.</figcaption></figure> <div class="mw-heading mw-heading2"><h2 id="Function">Function</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Glutathione_synthetase&action=edit&section=3" title="Edit section: Function"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Glutathione synthetase is important for a variety of biological functions in multiple organisms. In <i><a href="/wiki/Arabidopsis_thaliana" title="Arabidopsis thaliana">Arabidopsis thaliana</a></i>, low levels of glutathione synthetase have resulted in increased vulnerability to stressors such as <a href="/wiki/Toxic_heavy_metal" title="Toxic heavy metal">heavy metals</a>, toxic organic chemicals, and <a href="/wiki/Oxidative_stress" title="Oxidative stress">oxidative stress</a>.<sup id="cite_ref-Moyer_2010_15-0" class="reference"><a href="#cite_note-Moyer_2010-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> The presence of a <a href="/wiki/Thiol" title="Thiol">thiol</a> functional group allows its product GSH to serve both as an effective <a href="/wiki/Oxidizing_agent" title="Oxidizing agent">oxidizing</a> and <a href="/wiki/Reducing_agent" title="Reducing agent">reducing agent</a> in numerous biological scenarios. Thiols can easily accept a pair of electrons and become <a href="/wiki/Redox" title="Redox">oxidized</a> to <a href="/wiki/Disulfides" class="mw-redirect" title="Disulfides">disulfides</a>, and the disulfides can be readily <a href="/wiki/Redox" title="Redox">reduced</a> to regenerate thiols. Additionally, the thiol side chain of cysteines serve as potent <a href="/wiki/Nucleophiles" class="mw-redirect" title="Nucleophiles">nucleophiles</a> and react with oxidants and electrophilic species that would otherwise cause damage to the cell.<sup id="cite_ref-Xiang_2001_16-0" class="reference"><a href="#cite_note-Xiang_2001-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> Interactions with certain metals also stabilize thiolate intermediates.<sup id="cite_ref-Conte_2013_17-0" class="reference"><a href="#cite_note-Conte_2013-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </p><p>In humans, glutathione synthetase functions in a similar manner. Its product GSH participates in cellular pathways involved in homeostasis and cellular maintenance. For instance, <a href="/w/index.php?title=Glutathione_peroxidases&action=edit&redlink=1" class="new" title="Glutathione peroxidases (page does not exist)">glutathione peroxidases</a> catalyze the oxidation of GSH to <a href="/wiki/Glutathione_disulfide" title="Glutathione disulfide">glutathione disulfide</a> (GSSG) by reducing free radicals and reactive oxygen species such as hydrogen peroxide.<sup id="cite_ref-Suzuki_2002_18-0" class="reference"><a href="#cite_note-Suzuki_2002-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Glutathione_S-transferase" title="Glutathione S-transferase">Glutathione S-transferase</a> uses GSH to clean up various metabolites, xenobiotics, and electrophiles to <a href="/w/index.php?title=Mercapturates&action=edit&redlink=1" class="new" title="Mercapturates (page does not exist)">mercapturates</a> for excretion.<sup id="cite_ref-Fang_2002_19-0" class="reference"><a href="#cite_note-Fang_2002-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> Because of its antioxidant role, GSS mostly produce GSH inside the cytoplasm of liver cells and imported to mitochondria where detoxification occurs.<sup id="cite_ref-Ribas_2014_20-0" class="reference"><a href="#cite_note-Ribas_2014-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> GSH is also essential for the activation of the immune system to generate robust defense mechanisms against invading pathogens.<sup id="cite_ref-Fang_2002_19-1" class="reference"><a href="#cite_note-Fang_2002-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> GSH is capable of preventing infection from the influenza virus.<sup id="cite_ref-Townsend_2003_21-0" class="reference"><a href="#cite_note-Townsend_2003-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Cai_2003_22-0" class="reference"><a href="#cite_note-Cai_2003-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> </p> <figure class="mw-default-size mw-halign-center" typeof="mw:File/Thumb"><a href="/wiki/File:Gsh_mitochondria.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/d/d9/Gsh_mitochondria.jpg/310px-Gsh_mitochondria.jpg" decoding="async" width="310" height="197" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/d/d9/Gsh_mitochondria.jpg/465px-Gsh_mitochondria.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/d/d9/Gsh_mitochondria.jpg/620px-Gsh_mitochondria.jpg 2x" data-file-width="892" data-file-height="566" /></a><figcaption><a href="/wiki/Glutathione" title="Glutathione">Glutathione</a> (GSH) is synthesized in the cytoplasm of liver cells and imported to the <a href="/wiki/Mitochondria" class="mw-redirect" title="Mitochondria">mitochondria</a> where it functions as a <a href="/wiki/Cofactor_(biochemistry)" title="Cofactor (biochemistry)">cofactor</a> for a number of <a href="/wiki/Antioxidant" title="Antioxidant">antioxidant</a> and detoxifying enzymes.<sup id="cite_ref-Ribas_2014_20-1" class="reference"><a href="#cite_note-Ribas_2014-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup></figcaption></figure> <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=Glutathione_synthetase&action=edit&section=4" title="Edit section: Clinical significance"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Patients with mutations in the <i>GSS</i> gene develop <a href="/wiki/Glutathione_synthetase_deficiency" title="Glutathione synthetase deficiency">glutathione synthetase (GSS) deficiency</a>, an autosomal recessive disorder.<sup id="cite_ref-Ristoff_2001_23-0" class="reference"><a href="#cite_note-Ristoff_2001-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup> Patients develop a wide range of symptoms depending on the severity of the mutations. Mildly affected patients experience a compensated haemolytic anaemia because mutations affect stability of the enzyme. Moderately and severely affected individuals have enzymes with dysfunctional catalytic sites, rendering it unable to participate in detoxification reactions. Physiological symptoms include <a href="/wiki/Metabolic_acidosis" title="Metabolic acidosis">metabolic acidosis</a>, neurological defects, and increased susceptibility to pathogenic infections.<sup id="cite_ref-Njålsson_2005_4-1" class="reference"><a href="#cite_note-Njålsson_2005-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>Treatment of individuals with <a href="/wiki/Glutathione_synthetase_deficiency" title="Glutathione synthetase deficiency">glutathione synthetase deficiency</a> generally involve therapeutic treatments to address mild to severe symptoms and conditions. In order to treat <a href="/wiki/Metabolic_acidosis" title="Metabolic acidosis">metabolic acidosis</a>, severely affected patients are given large amounts of <a href="/wiki/Bicarbonate" title="Bicarbonate">bicarbonate</a> and <a href="/wiki/Antioxidants" class="mw-redirect" title="Antioxidants">antioxidants</a> such as <a href="/wiki/Vitamin_E" title="Vitamin E">vitamin E</a> and <a href="/wiki/Vitamin_C" title="Vitamin C">vitamin C</a>.<sup id="cite_ref-Kraut_2010_24-0" class="reference"><a href="#cite_note-Kraut_2010-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> In mild cases, <a href="/wiki/Ascorbate" class="mw-redirect" title="Ascorbate">ascorbate</a> and <a href="/wiki/Acetylcysteine" title="Acetylcysteine"><i>N</i>-acetylcysteine</a> have been shown to increase <a href="/wiki/Glutathione" title="Glutathione">glutathione</a> levels and increase <a href="/wiki/Erythrocyte" class="mw-redirect" title="Erythrocyte">erythrocyte</a> production.<sup id="cite_ref-Jain_1994_25-0" class="reference"><a href="#cite_note-Jain_1994-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> It is important to note that because glutathione synthetase deficiency is so rare, it is poorly understood. The disease also appears on a spectrum, so it is even more difficult to generalize among the few cases that occur.<sup id="cite_ref-Ristoff_2007_26-0" class="reference"><a href="#cite_note-Ristoff_2007-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Glutathione_synthetase&action=edit&section=5" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/Glutathione_synthetase_deficiency" title="Glutathione synthetase deficiency">Glutathione synthetase deficiency</a></li> <li><a href="/wiki/Glutathione" title="Glutathione">Glutathione</a></li> <li><a href="/wiki/Liver" title="Liver">Liver</a></li></ul> <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=Glutathione_synthetase&action=edit&section=6" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist reflist-columns references-column-width" style="column-width: 33em;"> <ol class="references"> <li id="cite_note-Gogos_2002-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-Gogos_2002_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="CITEREFGogosShapiro2002" class="citation journal cs1">Gogos A, Shapiro L (Dec 2002). <a rel="nofollow" class="external text" href="https://doi.org/10.1016%2FS0969-2126%2802%2900906-1">"Large conformational changes in the catalytic cycle of glutathione synthase"</a>. <i>Structure</i>. <b>10</b> (12): 1669–76. <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%2FS0969-2126%2802%2900906-1">10.1016/S0969-2126(02)00906-1</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/12467574">12467574</a>.</cite><span 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"Effect of ascorbate or N-acetylcysteine treatment in a patient with hereditary glutathione synthetase deficiency". <i>The Journal of Pediatrics</i>. <b>124</b> (2): 229–33. <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%2FS0022-3476%2894%2970309-4">10.1016/S0022-3476(94)70309-4</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/8301428">8301428</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=The+Journal+of+Pediatrics&rft.atitle=Effect+of+ascorbate+or+N-acetylcysteine+treatment+in+a+patient+with+hereditary+glutathione+synthetase+deficiency&rft.volume=124&rft.issue=2&rft.pages=229-33&rft.date=1994-02&rft_id=info%3Adoi%2F10.1016%2FS0022-3476%2894%2970309-4&rft_id=info%3Apmid%2F8301428&rft.aulast=Jain&rft.aufirst=A&rft.au=Buist%2C+NR&rft.au=Kennaway%2C+NG&rft.au=Powell%2C+BR&rft.au=Auld%2C+PA&rft.au=M%C3%A5rtensson%2C+J&rfr_id=info%3Asid%2Fen.wikipedia.org%3AGlutathione+synthetase" class="Z3988"></span></span> </li> <li id="cite_note-Ristoff_2007-26"><span class="mw-cite-backlink"><b><a href="#cite_ref-Ristoff_2007_26-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFRistoffLarsson2007" class="citation journal cs1">Ristoff E, Larsson A (2007). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1852094">"Inborn errors in the metabolism of glutathione"</a>. <i>Orphanet Journal of Rare Diseases</i>. <b>2</b>: 16. <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%2F1750-1172-2-16">10.1186/1750-1172-2-16</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/PMC1852094">1852094</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/17397529">17397529</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Orphanet+Journal+of+Rare+Diseases&rft.atitle=Inborn+errors+in+the+metabolism+of+glutathione&rft.volume=2&rft.pages=16&rft.date=2007&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1852094%23id-name%3DPMC&rft_id=info%3Apmid%2F17397529&rft_id=info%3Adoi%2F10.1186%2F1750-1172-2-16&rft.aulast=Ristoff&rft.aufirst=E&rft.au=Larsson%2C+A&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1852094&rfr_id=info%3Asid%2Fen.wikipedia.org%3AGlutathione+synthetase" class="Z3988"></span></span> </li> </ol></div> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFLawHalliwell1986" class="citation journal cs1">Law MY, Halliwell B (1986). "Purification and properties of glutathione synthetase from (Spinacia oleracea) leaves". <i>Plant Sci</i>. <b>43</b> (3): 185–191. <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%2F0168-9452%2886%2990016-6">10.1016/0168-9452(86)90016-6</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Plant+Sci.&rft.atitle=Purification+and+properties+of+glutathione+synthetase+from+%28Spinacia+oleracea%29+leaves&rft.volume=43&rft.issue=3&rft.pages=185-191&rft.date=1986&rft_id=info%3Adoi%2F10.1016%2F0168-9452%2886%2990016-6&rft.aulast=Law&rft.aufirst=MY&rft.au=Halliwell%2C+B&rfr_id=info%3Asid%2Fen.wikipedia.org%3AGlutathione+synthetase" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFMacnicol1987" class="citation journal cs1">Macnicol PK (1987). "Homoglutathione and glutathione synthetases of legume seedlings - partial-purification and substrate-specificity". <i>Plant Sci</i>. <b>53</b> (3): 229–235. <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/1987PlnSc..53..229M">1987PlnSc..53..229M</a>. <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%2F0168-9452%2887%2990159-2">10.1016/0168-9452(87)90159-2</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Plant+Sci.&rft.atitle=Homoglutathione+and+glutathione+synthetases+of+legume+seedlings+-+partial-purification+and+substrate-specificity&rft.volume=53&rft.issue=3&rft.pages=229-235&rft.date=1987&rft_id=info%3Adoi%2F10.1016%2F0168-9452%2887%2990159-2&rft_id=info%3Abibcode%2F1987PlnSc..53..229M&rft.aulast=Macnicol&rft.aufirst=PK&rfr_id=info%3Asid%2Fen.wikipedia.org%3AGlutathione+synthetase" class="Z3988"></span></li></ul> <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=Glutathione_synthetase&action=edit&section=7" title="Edit section: External links"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?name=Glutathione+Synthetase">Glutathione+Synthetase</a> at the U.S. National Library of Medicine <a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">Medical Subject Headings</a> (MeSH)</li></ul> <div class="navbox-styles"><style data-mw-deduplicate="TemplateStyles:r1129693374">.mw-parser-output .hlist dl,.mw-parser-output .hlist ol,.mw-parser-output .hlist ul{margin:0;padding:0}.mw-parser-output .hlist dd,.mw-parser-output .hlist dt,.mw-parser-output .hlist li{margin:0;display:inline}.mw-parser-output .hlist.inline,.mw-parser-output .hlist.inline dl,.mw-parser-output .hlist.inline ol,.mw-parser-output .hlist.inline ul,.mw-parser-output .hlist dl dl,.mw-parser-output .hlist dl ol,.mw-parser-output .hlist dl ul,.mw-parser-output .hlist ol dl,.mw-parser-output .hlist ol ol,.mw-parser-output .hlist ol ul,.mw-parser-output .hlist ul dl,.mw-parser-output .hlist ul ol,.mw-parser-output .hlist ul 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aria-labelledby="Metabolism:_Protein_metabolism,_synthesis_and_catabolism_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"><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar 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class="nv-edit"><a href="/wiki/Special:EditPage/Template:Amino_acid_metabolism_enzymes" title="Special:EditPage/Template:Amino acid metabolism enzymes"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Metabolism:_Protein_metabolism,_synthesis_and_catabolism_enzymes" style="font-size:114%;margin:0 4em"><a href="/wiki/Metabolism" title="Metabolism">Metabolism</a>: <a href="/wiki/Protein_metabolism" title="Protein metabolism">Protein metabolism</a>, <a href="/wiki/Amino_acid_synthesis" title="Amino acid synthesis">synthesis</a> and catabolism <a href="/wiki/Enzyme" title="Enzyme">enzymes</a></div></th></tr><tr><td class="navbox-abovebelow" colspan="2"><div><i><a href="/wiki/Essential_amino_acid" title="Essential amino acid">Essential amino acids</a> are in Capitals</i></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Ketogenic_amino_acid" title="Ketogenic amino acid">K</a>→<a href="/wiki/Acetyl-CoA" title="Acetyl-CoA">acetyl-CoA</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%"><a href="/wiki/Lysine" title="Lysine">LYSINE</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/Saccharopine_dehydrogenase" title="Saccharopine dehydrogenase">Saccharopine dehydrogenase</a></li> <li><a href="/wiki/Glutaryl-CoA_dehydrogenase" title="Glutaryl-CoA dehydrogenase">Glutaryl-CoA dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Leucine" title="Leucine">LEUCINE</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/3-Hydroxybutyryl-CoA_dehydrogenase" title="3-Hydroxybutyryl-CoA dehydrogenase">3-Hydroxybutyryl-CoA dehydrogenase</a></li> <li><a href="/wiki/Branched-chain_amino_acid_aminotransferase" title="Branched-chain amino acid aminotransferase">Branched-chain amino acid aminotransferase</a></li> <li><a href="/wiki/Branched-chain_alpha-keto_acid_dehydrogenase_complex" title="Branched-chain alpha-keto acid dehydrogenase complex">Branched-chain alpha-keto acid dehydrogenase complex</a></li> <li><a href="/wiki/Enoyl-CoA_hydratase" title="Enoyl-CoA hydratase">Enoyl-CoA hydratase</a></li> <li><a href="/wiki/3-hydroxy-3-methylglutaryl-CoA_lyase" class="mw-redirect" title="3-hydroxy-3-methylglutaryl-CoA lyase">HMG-CoA lyase</a></li> <li><a href="/wiki/HMG-CoA_reductase" title="HMG-CoA reductase">HMG-CoA reductase</a></li> <li><a href="/wiki/Isovaleryl_coenzyme_A_dehydrogenase" class="mw-redirect" title="Isovaleryl coenzyme A dehydrogenase">Isovaleryl coenzyme A dehydrogenase</a></li> <li><a href="/wiki/%CE%91-ketoisocaproate_dioxygenase" class="mw-redirect" title="Α-ketoisocaproate dioxygenase">α-Ketoisocaproate dioxygenase</a></li> <li><a href="/wiki/Leucine_2,3-aminomutase" title="Leucine 2,3-aminomutase">Leucine 2,3-aminomutase</a></li> <li><a href="/wiki/Methylcrotonyl-CoA_carboxylase" title="Methylcrotonyl-CoA carboxylase">Methylcrotonyl-CoA carboxylase</a></li> <li><a href="/wiki/Methylglutaconyl-CoA_hydratase" title="Methylglutaconyl-CoA hydratase">Methylglutaconyl-CoA hydratase</a></li></ul> <p>(See <a href="/wiki/Template:Leucine_metabolism_in_humans" title="Template:Leucine metabolism in humans">Template:Leucine metabolism in humans</a> – this diagram does not include the pathway for β-leucine synthesis via leucine 2,3-aminomutase) </p> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Tryptophan" title="Tryptophan">TRYPTOPHAN</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/Indoleamine_2,3-dioxygenase" title="Indoleamine 2,3-dioxygenase">Indoleamine 2,3-dioxygenase</a>/<a href="/wiki/Tryptophan_2,3-dioxygenase" title="Tryptophan 2,3-dioxygenase">Tryptophan 2,3-dioxygenase</a></li> <li><a href="/wiki/Arylformamidase" title="Arylformamidase">Arylformamidase</a></li> <li><a href="/wiki/Kynureninase" title="Kynureninase">Kynureninase</a></li> <li><a href="/wiki/3-hydroxyanthranilate_oxidase" title="3-hydroxyanthranilate oxidase">3-hydroxyanthranilate oxidase</a></li> <li><a href="/wiki/Aminocarboxymuconate-semialdehyde_decarboxylase" title="Aminocarboxymuconate-semialdehyde decarboxylase">Aminocarboxymuconate-semialdehyde decarboxylase</a></li> <li><a href="/wiki/Aminomuconate-semialdehyde_dehydrogenase" title="Aminomuconate-semialdehyde dehydrogenase">Aminomuconate-semialdehyde dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Phenylalanine" title="Phenylalanine">PHENYLALANINE</a>→<a href="/wiki/Tyrosine" title="Tyrosine">tyrosine</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>(see below)</li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Glucogenic_amino_acid" title="Glucogenic amino acid">G</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="G→pyruvate→citrate" scope="row" class="navbox-group" style="width:1%">G→<a href="/wiki/Pyruvate" class="mw-redirect" title="Pyruvate">pyruvate</a><br />→<a href="/wiki/Citrate" class="mw-redirect" title="Citrate">citrate</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Glycine" title="Glycine">glycine</a>→<a href="/wiki/Serine" title="Serine">serine</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/Serine_hydroxymethyltransferase" title="Serine hydroxymethyltransferase">Serine hydroxymethyltransferase</a></li> <li><a href="/wiki/Serine_dehydratase" title="Serine dehydratase">Serine dehydratase</a></li></ul> <ul><li><a href="/wiki/Glycine" title="Glycine">glycine</a>→<a href="/wiki/Creatine" title="Creatine">creatine</a>: <a href="/wiki/Guanidinoacetate_N-methyltransferase" title="Guanidinoacetate N-methyltransferase">Guanidinoacetate N-methyltransferase</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/Alanine" title="Alanine">alanine</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/Alanine_transaminase" title="Alanine transaminase">Alanine transaminase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Cysteine" title="Cysteine">cysteine</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/D-cysteine_desulfhydrase" title="D-cysteine desulfhydrase">D-cysteine desulfhydrase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Threonine" title="Threonine">threonine</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/L-threonine_dehydrogenase" class="mw-redirect" title="L-threonine dehydrogenase">L-threonine dehydrogenase</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">G→<a href="/wiki/Glutamate" class="mw-redirect" title="Glutamate">glutamate</a>→<br /><a href="/wiki/Alpha-Ketoglutaric_acid" class="mw-redirect" title="Alpha-Ketoglutaric acid">α-ketoglutarate</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Histidine" title="Histidine">HISTIDINE</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/Histidine_ammonia-lyase" title="Histidine ammonia-lyase">Histidine ammonia-lyase</a></li> <li><a href="/wiki/Urocanase" title="Urocanase">Urocanate hydratase</a></li> <li><a href="/wiki/Formiminotransferase_cyclodeaminase" class="mw-redirect" title="Formiminotransferase cyclodeaminase">Formiminotransferase cyclodeaminase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Proline" title="Proline">proline</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/Proline_oxidase" title="Proline oxidase">Proline oxidase</a></li> <li><a href="/wiki/Pyrroline-5-carboxylate_reductase" title="Pyrroline-5-carboxylate reductase">Pyrroline-5-carboxylate reductase</a></li> <li><a href="/wiki/1-Pyrroline-5-carboxylate_dehydrogenase" title="1-Pyrroline-5-carboxylate dehydrogenase">1-Pyrroline-5-carboxylate dehydrogenase</a>/<a href="/wiki/Aldehyde_dehydrogenase_4_family,_member_A1" title="Aldehyde dehydrogenase 4 family, member A1">ALDH4A1</a></li> <li><a href="/wiki/PYCR1" title="PYCR1">PYCR1</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Arginine" title="Arginine">arginine</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/Ornithine_aminotransferase" title="Ornithine aminotransferase">Ornithine aminotransferase</a></li> <li><a href="/wiki/Ornithine_decarboxylase" title="Ornithine decarboxylase">Ornithine decarboxylase</a></li> <li><a href="/wiki/Agmatinase" title="Agmatinase">Agmatinase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">→<a href="/wiki/Alpha-ketoglutarate" class="mw-redirect" title="Alpha-ketoglutarate">alpha-ketoglutarate</a>→TCA</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/Glutamate_dehydrogenase" title="Glutamate dehydrogenase">Glutamate dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Cysteine" title="Cysteine">cysteine</a>+<a href="/wiki/Glutamate" class="mw-redirect" title="Glutamate">glutamate</a>→<a href="/wiki/Glutathione" title="Glutathione">glutathione</a>: <a href="/wiki/Gamma-glutamylcysteine_synthetase" class="mw-redirect" title="Gamma-glutamylcysteine synthetase">Gamma-glutamylcysteine synthetase</a></li> <li><a class="mw-selflink selflink">Glutathione synthetase</a></li> <li><a href="/wiki/Gamma-glutamyl_transpeptidase" class="mw-redirect" title="Gamma-glutamyl transpeptidase">Gamma-glutamyl transpeptidase</a></li></ul> <ul><li><a href="/wiki/Glutamate" class="mw-redirect" title="Glutamate">glutamate</a>→<a href="/wiki/Glutamine" title="Glutamine">glutamine</a>: <a href="/wiki/Glutamine_synthetase" title="Glutamine synthetase">Glutamine synthetase</a></li> <li><a href="/wiki/Glutaminase" title="Glutaminase">Glutaminase</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">G→<a href="/wiki/Propionyl-CoA" title="Propionyl-CoA">propionyl-CoA</a>→<br /><a href="/wiki/Succinyl-CoA" title="Succinyl-CoA">succinyl-CoA</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Valine" title="Valine">VALINE</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/Branched-chain_amino_acid_aminotransferase" title="Branched-chain amino acid aminotransferase">Branched-chain amino acid aminotransferase</a></li> <li><a href="/wiki/Branched-chain_alpha-keto_acid_dehydrogenase_complex" title="Branched-chain alpha-keto acid dehydrogenase complex">Branched-chain alpha-keto acid dehydrogenase complex</a></li> <li><a href="/wiki/Enoyl-CoA_hydratase" title="Enoyl-CoA hydratase">Enoyl-CoA hydratase</a></li> <li><a href="/wiki/3-hydroxyisobutyryl-CoA_hydrolase" title="3-hydroxyisobutyryl-CoA hydrolase">3-hydroxyisobutyryl-CoA hydrolase</a></li> <li><a href="/wiki/3-hydroxyisobutyrate_dehydrogenase" title="3-hydroxyisobutyrate dehydrogenase">3-hydroxyisobutyrate dehydrogenase</a></li> <li><a href="/wiki/Methylmalonate-semialdehyde_dehydrogenase_(acylating)" title="Methylmalonate-semialdehyde dehydrogenase (acylating)">Methylmalonate semialdehyde dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Isoleucine" title="Isoleucine">ISOLEUCINE</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/Branched-chain_amino_acid_aminotransferase" title="Branched-chain amino acid aminotransferase">Branched-chain amino acid aminotransferase</a></li> <li><a href="/wiki/Branched-chain_alpha-keto_acid_dehydrogenase_complex" title="Branched-chain alpha-keto acid dehydrogenase complex">Branched-chain alpha-keto acid dehydrogenase complex</a></li> <li><a href="/wiki/3-hydroxy-2-methylbutyryl-CoA_dehydrogenase" title="3-hydroxy-2-methylbutyryl-CoA dehydrogenase">3-hydroxy-2-methylbutyryl-CoA dehydrogenase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Methionine" title="Methionine">METHIONINE</a>→</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/Methionine#Generation_of_homocysteine" title="Methionine">generation of homocysteine</a>:</i> <a href="/wiki/Methionine_adenosyltransferase" class="mw-redirect" title="Methionine adenosyltransferase">Methionine adenosyltransferase</a></li> <li><a href="/wiki/Adenosylhomocysteinase" title="Adenosylhomocysteinase">Adenosylhomocysteinase</a></li></ul> <ul><li><i><a href="/wiki/Methionine#Regeneration_of_methionine" title="Methionine">regeneration of methionine</a>:</i> <a href="/wiki/Methionine_synthase" title="Methionine synthase">Methionine synthase</a>/<a href="/wiki/Methionine_synthase" title="Methionine synthase">Homocysteine methyltransferase</a></li> <li><a href="/wiki/Betaine-homocysteine_S-methyltransferase" class="mw-redirect" title="Betaine-homocysteine S-methyltransferase">Betaine-homocysteine methyltransferase</a></li></ul> <ul><li><i><a href="/wiki/Methionine#Conversion_to_cysteine" title="Methionine">conversion to cysteine</a>:</i> <a href="/wiki/Cystathionine_beta_synthase" title="Cystathionine beta synthase">Cystathionine beta synthase</a></li> <li><a href="/wiki/Cystathionine_gamma-lyase" title="Cystathionine gamma-lyase">Cystathionine gamma-lyase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Threonine" title="Threonine">THREONINE</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/Threonine_aldolase" title="Threonine aldolase">Threonine aldolase</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">→<a href="/wiki/Succinyl-CoA" title="Succinyl-CoA">succinyl-CoA</a>→TCA</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/Propionyl-CoA_carboxylase" title="Propionyl-CoA carboxylase">Propionyl-CoA carboxylase</a></li> <li><a href="/wiki/Methylmalonyl_CoA_epimerase" title="Methylmalonyl CoA epimerase">Methylmalonyl CoA epimerase</a></li> <li><a href="/wiki/Methylmalonyl-CoA_mutase" title="Methylmalonyl-CoA mutase">Methylmalonyl-CoA mutase</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">G→<a href="/wiki/Fumarate" class="mw-redirect" title="Fumarate">fumarate</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Phenylalanine" title="Phenylalanine">PHENYLALANINE</a>→<a href="/wiki/Tyrosine" title="Tyrosine">tyrosine</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/Phenylalanine_hydroxylase" title="Phenylalanine hydroxylase">Phenylalanine hydroxylase</a></li> <li><a href="/wiki/Tyrosine_aminotransferase" title="Tyrosine aminotransferase">Tyrosine aminotransferase</a></li> <li><a href="/wiki/4-Hydroxyphenylpyruvate_dioxygenase" title="4-Hydroxyphenylpyruvate dioxygenase">4-Hydroxyphenylpyruvate dioxygenase</a></li> <li><a href="/wiki/Homogentisate_1,2-dioxygenase" title="Homogentisate 1,2-dioxygenase">Homogentisate 1,2-dioxygenase</a></li> <li><a href="/wiki/Fumarylacetoacetate_hydrolase" title="Fumarylacetoacetate hydrolase">Fumarylacetoacetate hydrolase</a></li></ul> <ul><li><a href="/wiki/Tyrosine" title="Tyrosine">tyrosine</a>→<a href="/wiki/Melanin" title="Melanin">melanin</a>: <a href="/wiki/Tyrosinase" title="Tyrosinase">Tyrosinase</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">G→<a href="/wiki/Oxaloacetic_acid" title="Oxaloacetic acid">oxaloacetate</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Asparagine" title="Asparagine">asparagine</a>→<a href="/wiki/Aspartate" class="mw-redirect" title="Aspartate">aspartate</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/Asparaginase" title="Asparaginase">Asparaginase</a>/<a href="/wiki/Asparagine_synthetase" title="Asparagine synthetase">Asparagine synthetase</a></li> <li><a href="/wiki/Aspartate_transaminase" title="Aspartate transaminase">Aspartate transaminase</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr></tbody></table><div></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:_CO_CS_and_CN_ligases_(EC_6.1-6.3)" 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:Ligases_CO_CS_and_CN" title="Template:Ligases CO CS and CN"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Ligases_CO_CS_and_CN" title="Template talk:Ligases CO CS and CN"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Ligases_CO_CS_and_CN" title="Special:EditPage/Template:Ligases CO CS and CN"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Enzymes:_CO_CS_and_CN_ligases_(EC_6.1-6.3)" style="font-size:114%;margin:0 4em"><a href="/wiki/Enzyme" title="Enzyme">Enzymes</a>: CO CS and CN <a href="/wiki/Ligase" title="Ligase">ligases</a> (<a href="/wiki/Enzyme_Commission_number" title="Enzyme Commission number">EC</a> 6.1-6.3)</div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/List_of_EC_numbers_(EC_6)#EC_6.1:_Forming_Carbon-Oxygen_Bonds" title="List of EC numbers (EC 6)">6.1</a>: Carbon-Oxygen</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/Aminoacyl_tRNA_synthetase" title="Aminoacyl tRNA synthetase">Aminoacyl tRNA synthetase</a> <ul><li><a href="/wiki/Alanine%E2%80%94tRNA_ligase" title="Alanine—tRNA ligase">Alanine</a></li> <li><a href="/wiki/Arginine%E2%80%94tRNA_ligase" title="Arginine—tRNA ligase">Arginine</a></li> <li><a href="/wiki/Asparagine%E2%80%94tRNA_ligase" title="Asparagine—tRNA ligase">Asparagine</a></li> <li><a href="/wiki/Aspartate%E2%80%94tRNA_ligase" title="Aspartate—tRNA ligase">Aspartate</a></li> <li><a href="/wiki/Cysteine%E2%80%94tRNA_ligase" title="Cysteine—tRNA ligase">Cysteine</a></li> <li><a href="/wiki/D-alanine%E2%80%94poly(phosphoribitol)_ligase" title="D-alanine—poly(phosphoribitol) ligase">D-alanine—poly(phosphoribitol) ligase</a></li> <li><a href="/wiki/Glutamate%E2%80%94tRNA_ligase" title="Glutamate—tRNA ligase">Glutamate</a></li> <li><a href="/wiki/Glutamine%E2%80%94tRNA_ligase" title="Glutamine—tRNA ligase">Glutamine</a></li> <li><a href="/wiki/Glycine%E2%80%94tRNA_ligase" title="Glycine—tRNA ligase">Glycine</a></li> <li><a href="/wiki/Histidine%E2%80%94tRNA_ligase" title="Histidine—tRNA ligase">Histidine</a></li> <li><a href="/wiki/Isoleucine%E2%80%94tRNA_ligase" title="Isoleucine—tRNA ligase">Isoleucine</a></li> <li><a href="/wiki/Leucine%E2%80%94tRNA_ligase" title="Leucine—tRNA ligase">Leucine</a></li> <li><a href="/wiki/Lysine%E2%80%94tRNA_ligase" title="Lysine—tRNA ligase">Lysine</a></li> <li><a href="/wiki/Methionine%E2%80%94tRNA_ligase" title="Methionine—tRNA ligase">Methionine</a></li> <li><a href="/wiki/Phenylalanine%E2%80%94tRNA_ligase" title="Phenylalanine—tRNA ligase">Phenylalanine</a></li> <li><a href="/wiki/Proline%E2%80%94tRNA_ligase" title="Proline—tRNA ligase">Proline</a></li> <li><a href="/wiki/Serine%E2%80%94tRNA_ligase" title="Serine—tRNA ligase">Serine</a></li> <li><a href="/wiki/Threonine%E2%80%94tRNA_ligase" title="Threonine—tRNA ligase">Threonine</a></li> <li><a href="/wiki/Tryptophan%E2%80%94tRNA_ligase" title="Tryptophan—tRNA ligase">Tryptophan</a></li> <li><a href="/wiki/Tyrosine%E2%80%94tRNA_ligase" title="Tyrosine—tRNA ligase">Tyrosine</a></li> <li><a href="/wiki/Valine%E2%80%94tRNA_ligase" title="Valine—tRNA ligase">Valine</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_6)#EC_6.2:_Forming_Carbon-Sulfur_Bonds" title="List of EC numbers (EC 6)">6.2</a>: Carbon-Sulfur</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/Succinyl_coenzyme_A_synthetase" title="Succinyl coenzyme A synthetase">Succinyl coenzyme A synthetase</a></li> <li><a href="/wiki/Acetyl%E2%80%94CoA_synthetase" class="mw-redirect" title="Acetyl—CoA synthetase">Acetyl—CoA synthetase</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%"><a href="/wiki/List_of_EC_numbers_(EC_6)#EC_6.3:_Forming_Carbon-Nitrogen_Bonds" title="List of EC numbers (EC 6)">6.3</a>: Carbon-Nitrogen</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/Glutamine_synthetase" title="Glutamine synthetase">Glutamine synthetase</a></li> <li><a href="/wiki/Ubiquitin_ligase" title="Ubiquitin ligase">Ubiquitin ligase</a> <ul><li><a href="/wiki/Cullin" title="Cullin">Cullin</a></li> <li><a href="/wiki/Von_Hippel%E2%80%93Lindau_tumor_suppressor" title="Von Hippel–Lindau tumor suppressor">Von Hippel–Lindau tumor suppressor</a></li> <li><a href="/wiki/UBE3A" title="UBE3A">UBE3A</a></li> <li><a href="/wiki/Mdm2" title="Mdm2">Mdm2</a></li> <li><a href="/wiki/Anaphase-promoting_complex" title="Anaphase-promoting complex">Anaphase-promoting complex</a></li> <li><a href="/wiki/UBR1" title="UBR1">UBR1</a></li></ul></li> <li><a class="mw-selflink selflink">Glutathione synthetase</a></li> <li><a href="/wiki/CTP_synthetase" title="CTP synthetase">CTP synthetase</a></li> <li><a href="/wiki/Adenylosuccinate_synthase" title="Adenylosuccinate synthase">Adenylosuccinate synthase</a></li> <li><a href="/wiki/Argininosuccinate_synthase" title="Argininosuccinate synthase">Argininosuccinate synthase</a></li> <li><a href="/wiki/Holocarboxylase_synthetase" title="Holocarboxylase synthetase">Holocarboxylase synthetase</a></li> <li><a href="/wiki/GMP_synthase_(glutamine%E2%80%94hydrolysing)" class="mw-redirect" title="GMP synthase (glutamine—hydrolysing)">GMP synthase</a></li> <li><a href="/wiki/Asparagine_synthetase" title="Asparagine synthetase">Asparagine synthetase</a></li> <li><a href="/wiki/Carbamoyl_phosphate_synthetase" title="Carbamoyl phosphate synthetase">Carbamoyl phosphate synthetase</a> <ul><li><a href="/wiki/Carbamoyl_phosphate_synthetase_I" title="Carbamoyl phosphate synthetase I">I</a></li> <li><a href="/wiki/Carbamoyl_phosphate_synthase_II" title="Carbamoyl phosphate synthase II">II</a></li></ul></li> <li><a href="/wiki/Glutamate%E2%80%93cysteine_ligase" title="Glutamate–cysteine ligase">Glutamate–cysteine ligase</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" 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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 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