beta-methylglutaconyl-CoA + ADP + orthophosphate beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA)

bMG-CoA + ADP + Pi + H+ → bMC-CoA + ATP + HCO3-

Reactome

R-HSA-508308

Compartment

mitochondrial matrix

Rhea

RHEA:13591

Catalysed by

• methylcrotonoyl-CoA carboxylase activity of 6x(Btn-MCCC1:MCCC2) [mitochondrial matrix]EC 6.4.1.4

Literature

1. The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. J Clin Invest, 2001
2. Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency. Hum Mol Genet, 2001