{"metadata":{"accession":"A0A6B0RCX1","id":"A0A6B0RCX1_9CETA","source_organism":{"taxId":"72004","scientificName":"Bos mutus","fullName":"Bos mutus (wild yak)"},"name":"Methylmalonic aciduria type A protein, mitochondrial","description":["GTPase, binds and hydrolyzes GTP. Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis. Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MMUT). Plays a dual role as both a protectase and a reactivase for MMUT. Protects MMUT from progressive inactivation by oxidation by decreasing the rate of the formation of the oxidized inactive cofactor hydroxocobalamin (OH2Cbl). Additionally acts a reactivase by promoting the replacement of OH2Cbl by the active cofactor AdoCbl, restoring the activity of MMUT in the presence and hydrolysis of GTP"],"length":421,"sequence":"MNIPMLLQRPHQHFLKGLVRTPFRCYRFIFHPSPHLGSGVPCAQPLSSPGLHCAKWMLPSNGLTRKLCLQTTLEEHTEGLSDKEQRLVDKLYAGLIQGQRACLAEAITLVESTHSRKKELAQVLLQKVLAHHREQENLNKGKPLAFRVGLSGPPGAGKSTFIEYFGKMLTERGHKLSVLAVDPSSCTSGGSLLGDKTRMTELSRDMNAYIRPSPTRGTLGGVTRTTNEAILLCEGGGYDIILVETVGVGQSEFAVADMVDMFILLLPPAGGDELQGIKRGIVEMADLVAITKSDGDLIVPARRIQAEYVSALKLLRRRSGVWRPKVIRISARSGEGITEMWDKMKEFQEVMLVSGELAAKRQKQQKVWMWNLIQENVIEHFRTHPAVREQIPLLEKRVLSGALSPGLAADLLLKAFKSRHE","proteome":"UP000322234","gene":"E5288_WYG016375","go_terms":[{"identifier":"GO:0003924","name":"GTPase activity","category":{"code":"F","name":"molecular_function"}},{"identifier":"GO:0005525","name":"GTP binding","category":{"code":"F","name":"molecular_function"}}],"protein_evidence":3,"source_database":"unreviewed","is_fragment":false,"in_alphafold":true,"in_bfvd":false,"ida_accession":"55403b17a9be0643383e4d6e5af8f55858d8be16","counters":{"domain_architectures":15093,"entries":11,"isoforms":0,"proteomes":1,"sets":2,"structures":0,"taxa":1,"dbEntries":{"cathgene3d":3,"ssf":1,"cdd":1,"pfam":1,"panther":1,"ncbifam":2,"interpro":2},"proteome":1,"taxonomy":1,"similar_proteins":15093}}}