Reactome | beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA]

beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA]

Stable Identifier

R-HSA-70773

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA]

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Methylcrotonyl CoA carboxylase (MCCA) catalyzes the reversible reaction of beta-methylcrotonyl-CoA, ATP, and CO2 to form beta-methylglutaconyl-CoA, ADP, orthophosphate, and H2O. Active MCCA is composed of two polypeptides, MCCA1 and MCCA2 (Baumgartner et al. 2001; Holzinger et al. 2001). The enzyme has been purified from fibroblast mitochondria. By analogy to the more thoroughly studied bovine homologue, MCCA is thought to be a hexamer of six MCCA1:MCCA2 dimers, and the MCCA1 polypeptide is thought to have a biotin moiety covalently bound to lysine residue 681. Localization of the complex to the mitochondrial inner membrane is inferred from studies of the bovine homologue (Hector et al. 1980). Mitochondrial import of MCCA1 and 2 is associated with removal of aminoterminal mitochondrial targeting sequences (Stadler et al. 2005).

Literature References

PubMed ID	Title	Journal	Year
7356336	Subcellular localization of 3-methylcrotonyl-coenzyme A carboxylase in bovine kidney Hector, ML, Logue, EA, Cochran, BC, Fall, RR	Arch Biochem Biophys	1980
11406611	Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency. Holzinger, A, Röschinger, W, Roscher, AA, Koch, HG, Muntau, AC, Thuy, LP, Nyhan, WL, Lichtner, P, Kattenfeld, T, Lagler, F, Mayerhofer, PU	Hum Mol Genet	2001
11181649	The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. Baumgartner, ER, Cole, RN, Packman, S, Obie, C, Baumgartner, MR, Suormala, T, Valle, D, Almashanu, S	J Clin Invest	2001
16023992	Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase Holzinger, A, Anslinger, K, Röschinger, W, Roscher, AA, Herrmann, JM, Polanetz, R, Stadler, SC, Meier, S, Mayerhofer, PU	Biochem Biophys Res Commun	2005