{"entryType":"UniProtKB reviewed (Swiss-Prot)","primaryAccession":"O96033","uniProtkbId":"MOC2A_HUMAN","entryAudit":{"firstPublicDate":"2002-02-11","lastAnnotationUpdateDate":"2026-09-02","lastSequenceUpdateDate":"1999-05-01","entryVersion":178,"sequenceVersion":1},"annotationScore":5.0,"organism":{"scientificName":"Homo sapiens","commonName":"Human","taxonId":9606,"lineage":["Eukaryota","Metazoa","Chordata","Craniata","Vertebrata","Euteleostomi","Mammalia","Eutheria","Euarchontoglires","Primates","Haplorrhini","Catarrhini","Hominidae","Homo"]},"proteinExistence":"1: Evidence at protein level","proteinDescription":{"recommendedName":{"fullName":{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"Molybdopterin synthase sulfur carrier subunit"}},"alternativeNames":[{"fullName":{"value":"MOCO1-A"}},{"fullName":{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"Molybdenum cofactor synthesis protein 2 small subunit"}},{"fullName":{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"Molybdenum cofactor synthesis protein 2A"},"shortNames":[{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"MOCS2A"}]},{"fullName":{"value":"Molybdopterin-synthase small subunit"}},{"fullName":{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"Sulfur carrier protein MOCS2A"}}]},"genes":[{"geneName":{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"MOCS2"},"synonyms":[{"value":"MOCO1"}]}],"comments":[{"texts":[{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12732628"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"22453920"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"25709896"}],"value":"Acts as a sulfur carrier required for molybdopterin biosynthesis (PubMed:22453920). Component of the molybdopterin synthase complex that catalyzes the conversion of precursor Z into molybdopterin by mediating the incorporation of 2 sulfur atoms into precursor Z to generate a dithiolene group. In the complex, serves as sulfur donor by being thiocarboxylated (-COSH) at its C-terminus by MOCS3 (PubMed:25709896, PubMed:12732628, PubMed:22453920). After interaction with MOCS2B, the sulfur is then transferred to precursor Z to form molybdopterin (PubMed:22453920)"}],"commentType":"FUNCTION"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"Cofactor biosynthesis; molybdopterin biosynthesis"}],"commentType":"PATHWAY"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12732628"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"25709896"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"26705305"}],"value":"Heterotetramer; composed of 2 small (MOCS2A) and 2 large (MOCS2B) subunits (By similarity) (PubMed:12732628). Component of the molybdopterin synthase complex consisting of MOCS2A and MOCS2B (PubMed:12732628, PubMed:25709896, PubMed:26705305). Interacts with the histone acetyltransferase ATAC complex component MBIP (PubMed:26705305)"}],"commentType":"SUBUNIT"},{"commentType":"INTERACTION","interactions":[{"interactantOne":{"uniProtKBAccession":"O96033","intActId":"EBI-9056334"},"interactantTwo":{"uniProtKBAccession":"O96007","geneName":"MOCS2","intActId":"EBI-723640"},"numberOfExperiments":2,"organismDiffer":false}]},{"commentType":"SUBCELLULAR LOCATION","subcellularLocations":[{"location":{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15073332"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"22453920"}],"value":"Cytoplasm, cytosol","id":"SL-0091"}},{"location":{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"22453920"}],"value":"Nucleus","id":"SL-0191"}}]},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10053003"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"9889283"}],"value":"Widely expressed. Highest levels are found in heart and skeletal muscle. Lower levels are present in brain, kidney and pancreas. Very low levels are found in lung and peripheral blood leukocytes"}],"commentType":"TISSUE SPECIFICITY"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12732628"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15073332"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15910006"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"17459099"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18491921"}],"value":"C-terminal thiocarboxylation occurs in 2 steps, it is first acyl-adenylated (-COAMP) via the hesA/moeB/thiF part of MOCS3, then thiocarboxylated (-COSH) via the rhodanese domain of MOCS3"}],"commentType":"PTM"},{"commentType":"DISEASE","disease":{"diseaseId":"Molybdenum cofactor deficiency, type B1","diseaseAccession":"DI-01990","acronym":"MOCODB1","description":"An autosomal recessive metabolic disorder characterized by neonatal onset of intractable seizures, opisthotonus, and facial dysmorphism associated with hypouricemia and elevated urinary sulfite levels. 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Expression of these 2 proteins are related since a mutation that removes the start codon of the small subunit (MOCS2A) also impairs expression of the large subunit (MOCS2B)"}],"commentType":"MISCELLANEOUS"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"}],"value":"Belongs to the MoaD family. MOCS2A subfamily"}],"commentType":"SIMILARITY"}],"features":[{"type":"Chain","location":{"start":{"value":1,"modifier":"EXACT"},"end":{"value":88,"modifier":"EXACT"}},"description":"Molybdopterin synthase sulfur carrier subunit","featureId":"PRO_0000209134"},{"type":"Modified residue","location":{"start":{"value":88,"modifier":"EXACT"},"end":{"value":88,"modifier":"EXACT"}},"description":"1-thioglycine; alternate","evidences":[{"evidenceCode":"ECO:0000255","source":"HAMAP-Rule","id":"MF_03051"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12732628"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15073332"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15910006"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"17459099"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18491921"},{"evidenceCode":"ECO:0000305","source":"PubMed","id":"22453920"}]},{"type":"Modified 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