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

Stable Identifier
R-HSA-70773
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
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, Lagler, F, Mayerhofer, PU, Lichtner, P, Kattenfeld, T, Thuy, LP, Nyhan, WL, Koch, HG, Muntau, AC, Roscher, AA

Hum Mol Genet 2001
7356336 Subcellular localization of 3-methylcrotonyl-coenzyme A carboxylase in bovine kidney

Hector, ML, Cochran, BC, Logue, EA, Fall, RR

Arch Biochem Biophys 1980
11181649 The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency.

Baumgartner, MR, Almashanu, S, Suormala, T, Obie, C, Cole, RN, Packman, S, Baumgartner, ER, Valle, D

J Clin Invest 2001
16023992 Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase

Stadler, SC, Polanetz, R, Meier, S, Mayerhofer, PU, Herrmann, JM, Anslinger, K, Roscher, AA, Röschinger, W, Holzinger, A

Biochem Biophys Res Commun 2005
Participants
Participant Of
Event Information
Catalyst Activity
Catalyst Activity
Title
methylcrotonoyl-CoA carboxylase activity of 6x(Btn-MCCC1:MCCC2) [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea