Gene Symbol | MCCC2 |
---|---|
Entrez Gene | 64087 |
Alt Symbol | MCCB |
Species | Human |
Gene Type | protein-coding |
Description | methylcrotonoyl-CoA carboxylase 2 (beta) |
Other Description | 3-methylcrotonyl-CoA carboxylase 2|3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit|3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta|MCCase subunit beta|biotin carboxylase|methylcrotonoyl-CoA carboxylase beta chain, mitochondrial|methylcrotonoyl-Coenzyme A carboxylase 2 (beta)|non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase |
Swissprots | Q9Y4L7 Q96C27 Q9HCC0 A6NIY9 |
Accessions | BAB41121 EAW95691 EAW95692 EAW95693 Q9HCC0 AB050049 BAB16880 AF261884 AAK49409 AF301000 AAK16404 AF310971 AAG53094 AK025591 AK090834 BAG52236 AK310194 AL079298 CAB45194 BC014897 AAH14897 BC065027 AAH65027 CN309723 XM_005248567 XP_005248624 XM_011543528 XP_011541830 XM_011543529 XP_011541831 NM_022132 NP_071415 |
Function | Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. {ECO:0000269|PubMed:17360195}. |
Subcellular Location | Mitochondrion matrix {ECO:0000269|PubMed:11170888, ECO:0000269|PubMed:16023992}. |
Top Pathways | Valine, leucine and isoleucine degradation |