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

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

Stable Identifier

R-HSA-71031

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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

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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		2001
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