Methylcrotonyl-CoA carboxylase
| Methylcrotonyl-CoA carboxylase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC no. | 6.4.1.4 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| |||||||||
| Methylcrotonoyl-coenzyme A carboxylase 1 (alpha) | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | MCCC1 | ||||||
| NCBI gene | 56922 | ||||||
| HGNC | 6936 | ||||||
| OMIM | 609010 | ||||||
| RefSeq | NM_020166 | ||||||
| UniProt | Q96RQ3 | ||||||
| Other data | |||||||
| EC number | 6.4.1.4 | ||||||
| Locus | Chr. 3 q27.1 | ||||||
| |||||||
| Methylcrotonoyl-coenzyme A carboxylase 2 (beta) | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | MCCC2 | ||||||
| NCBI gene | 64087 | ||||||
| HGNC | 6937 | ||||||
| OMIM | 609014 | ||||||
| RefSeq | NM_022132 | ||||||
| UniProt | Q9HCC0 | ||||||
| Other data | |||||||
| EC number | 6.4.1.4 | ||||||
| Locus | Chr. 5 q12-q13 | ||||||
| |||||||
Methylcrotonyl CoA carboxylase (EC 6.4.1.4, MCC) (3-methylcrotonyl CoA carboxylase, methylcrotonoyl-CoA carboxylase) is a biotin-requiring enzyme located in the mitochondria. MCC uses bicarbonate as a carboxyl group source to catalyze the carboxylation of a carbon adjacent to a carbonyl group performing the fourth step in processing leucine, an essential amino acid.