ENTRY       EC 6.4.1.3
NAME        Propionyl-CoA carboxylase
CLASS       Ligases
            Forming carbon-carbon bonds
SYSNAME     Propanoyl-CoA:carbon-dioxide ligase (ADP-forming)
REACTION    ATP + Propanoyl-CoA + HCO3- = ADP + Orthophosphate +
            (S)-Methylmalonyl-CoA
SUBSTRATE   ATP
            Propanoyl-CoA
            Butanoyl-CoA
            HCO3-
PRODUCT     ADP
            Orthophosphate
            (S)-Methylmalonyl-CoA
COFACTOR    Biotin
COMMENT     A biotinyl-protein. Also carboxylates butanoyl-CoA and catalyses
            transcarboxylation.
PATHWAY     PATH: MAP00280  Valine, leucine and isoleucine degradation
            PATH: MAP00640  Propanoate metabolism
DISEASE     MIM: 232000  Propionyl Coenzyme A carboxylase, alpha polypeptide;
                         Propionicacidemia, type I or pccA type (1)
            MIM: 232050  Propionyl Coenzyme A carboxylase, beta polypeptide;
                         Propionicacidemia, type II or pccB type (3)
MOTIF       PS: PS00188  [GN]-[DEQ]-x-[LIVMFY]-x(2)-[LIVM]-x-[AIV]-M-K-[LMA]-
                         x(3)-[LIVM]-x-[SA]
            PS: PS00866  [FYV]-P-[LIVMC]-[LIVMA]-[LIVM]-[KR]-[PSA]-[STA]-x(3)-
                         [SG]-G-x-[AG]
            PS: PS00867  [LIVMF]-[LIMN]-E-[LIVMCA]-N-[PATLIVM]-[KR]-[LIVMSTAC]
GENES       BSU: yqjD
            HSA: PCCA(Hs.80741) PCCB(Hs.63788)
DBLINKS     University of Geneva ENZYME DATA BANK: 6.4.1.3
            WIT (What Is There) Metabolic Reconstruction: 6.4.1.3
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