Propionyl-CoA carboxylase

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This article deals with molecular science, for other meanings of the acronym PCCA, see here.
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

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[1] and a lyase.[2]

Contents

[edit] Pathology

A deficiency is associated with propionic acidemia.

[edit] See also

[edit] References

  1. ^ EC 6.4.1.3
  2. ^ EC 4.1.1.41

[edit] External links

v • d • e
Ligases: carbon-carbon ligases (EC 6.4)
v • d • e
Metabolism: lipid metabolism - triglyceride and fatty acid enzymes
Lipid production
Lipid degradation
see also disorders
v • d • e
Metabolism: amino acid metabolism - synthesis and catabolism enzymes (essential in CAPS)
K→acetyl-CoA
(see below)
G→pyruvate
G→glutamate
G→propionyl-CoA
→succinyl-CoA→TCA
Propionyl-CoA carboxylase - Methylmalonyl-CoA mutase
G→fumarate
G→oxaloacetate
Other
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