Propionyl-CoA carboxylase

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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

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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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[1] and a lyase.[2]

Contents

Pathology

A deficiency is associated with propionic acidemia.

See also

References

  1. EC 6.4.1.3
  2. EC 4.1.1.41

External links


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