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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

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]

Pathology

A deficiency is associated with propionic acidemia.

See also

References

  1. ^ EC 6.4.1.3
  2. ^ EC 4.1.1.41
 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Propionyl-CoA_carboxylase". A list of authors is available in Wikipedia.
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