beta-methylcrotonyl-CoA + ATP + HCO3- beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
bMC-CoA + ATP + HCO3- → bMG-CoA + ADP + Pi + H+
- Reactome
- R-HSA-70773
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:13590
Inputs
- bMC-CoACHEBI:57344, C26H38N7O17P3S, mitochondrial matrix
- ATPCHEBI:30616, C10H12N5O13P3, mitochondrial matrix
- HCO3-CHEBI:17544, CHO3, mitochondrial matrix
Outputs
- bMG-CoACHEBI:57346, C27H37N7O19P3S, mitochondrial matrix
- ADPCHEBI:456216, C10H12N5O10P2, mitochondrial matrix
- PiCHEBI:43474, HO4P, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Catalysed by
- methylcrotonoyl-CoA carboxylase activity of 6x(Btn-MCCC1:MCCC2) [mitochondrial matrix]EC 6.4.1.4
Literature
- The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. J Clin Invest, 2001
- Subcellular localization of 3-methylcrotonyl-coenzyme A carboxylase in bovine kidney Arch Biochem Biophys, 1980
- 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
- Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase Biochem Biophys Res Commun, 2005