Propionyl-CoA carboxylase

propionyl Coenzyme A carboxylase, alpha polypeptide
Identifiers
Symbol	PCCA
Entrez	5095
HUGO	8653
OMIM	232000
RefSeq	NM_000282
UniProt	P05165
Other data
EC number	6.4.1.3
Locus	Chr. 13 q32

propionyl Coenzyme A carboxylase, beta polypeptide
Identifiers
Symbol	PCCB
Entrez	5096
HUGO	8654
OMIM	232050
RefSeq	NM_000532
UniProt	P05166
Other data
EC number	6.4.1.3
Locus	Chr. 3 q21-q22

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Propionyl-CoA carboxylase catalyses the carboxylation reaction of propionyl CoA. The product of the reaction is D-methylmalonyl CoA.

 ATP + propanoyl-CoA + HCO3- <=> ADP + phosphate + (S)-methylmalonyl-CoA

It has been classified both as a ligase and a lyase.

Pathology

A deficiency is associated with propionic acidemia.

See also

• Anaplerotic reactions

External links

• Propionyl-CoA+Carboxylase at the US National Library of Medicine Medical Subject Headings (MeSH)