8jxl

Structural highlights

Disease

MCCB_HUMAN 3-methylcrotonyl-CoA carboxylase deficiency. The disease is caused by variants affecting the gene represented in this entry.

Function

MCCB_HUMAN 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.^[1]

References

1. ↑ Chu CH, Cheng D. Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC). Protein Expr Purif. 2007 Jun;53(2):421-7. PMID:17360195 doi:10.1016/j.pep.2007.01.012