propionyl-CoA + CO2 + ATP <=> D-methylmalonyl-CoA + ADP + orthophosphate

Stable Identifier
Reaction [transition]
Homo sapiens
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Propionyl CoA carboxylase in the mitochondrial matrix catalyzes the reaction of propionyl-CoA, CO2, and ATP to form D-methylmalonyl-CoA, ADP, and orthophosphate. The active form of the enzyme is a heteromultimer, probably consisting of six alpha subunits each bound to a biotin molecule and six beta subunits (Kaziro et al. 1961; Kalousek et al. 1980; Fenton et al. 2001). Both alpha and beta subunits are posttranslationally modified to remove amino-terminal mitochondrial import sequences (Stadler et al. 2005).

Literature References
PubMed ID Title Journal Year
  The Metabolic and Molecular Bases of Inherited Disease, 8th ed

Beaudet, AL, Scriver, CR, Sly, WS, Valle, D

6765947 Isolation and characterization of propionyl-CoA carboxylase from normal human liver. Evidence for a protomeric tetramer of nonidentical subunits

Kalousek, F, Darigo, MD, Rosenberg, LE

J Biol Chem 1980
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
13752080 Metabolism of propionic acid in animal tissues. VIII. Crystalline propionyl carboxylase

Ochoa, S, Kaziro, Y, Warner, RC, Chen, JY

J Biol Chem 1961
Catalyst Activity

propionyl-CoA carboxylase activity of 6x(Btn-PCCA:PCCB) [mitochondrial matrix]

Orthologous Events
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