Pcca
entrez
687008
canonical
uniprot
P14882
canonical
ensembl
ENSRNOG00000057042
resolver
ensp
ENSRNOP00000072844
resolver
ensp
ENSRNOP00000076923
resolver
ensp
ENSRNOP00000082095
resolver
enst
ENSRNOT00000077482
resolver
enst
ENSRNOT00000094922
resolver
genesymbol
Pcca
resolver
uniprot
A0A0G2K401
resolver
uniprot
A0A8I5ZWE0
resolver
uniprot_entry
A0A0G2K401_RAT
resolver
uniprot_entry
A0A8I5ZWE0_RAT
resolver
uniprot_entry
PCCA_RAT
resolver
amino_acid_sequence
MAGLWVRTVALLAARRHWRRSSQQLLWTLKRAPRSSQQLLWTLKRAPVYSQQCLVVSRSLSSVEYEPKEKTFDKILIANRGEIACRVIKTCRKMGIRTVAIHSDVDASSVHVKMADEAVCVGPAPTSKSYLNMDAIMEAIKKTGAQAVHPGYGFLSENKEFAKCLAAEDVTFIGPDTHAIQAMGDKIESKLLAKRAKVNTIPGFDGVLKDADEAVRIAREIGYPVMIKASAGGGGKGMRIPWDDEETRDGFRFSSQEAASSFGDDRLLIEKFIDNPRHIEIQVLGDKHGNALWLNERECSIQRRNQKVVEEAPSIFLDPETRRAMGEQAVAWPKAVKYSSAGTVEFLVDSQKNFYFLEMNTRLQVEHPVTECITGLDLVQEMILVAKGYPLRHKQEDIPISGWAVECRVYAEDPYKSFGLPSIGRLSQYQEPIHLPGVRVDSGIQPGSDISIYHDPMISKLVTYGSDRAEALKRMEDALDSYVIRGVTHNIPLLREVIINTRFVKGDISTKFLSDVYPDGFKGHMLTPSERDQLLAIASSLFVASQLRAQRFQEHSRVPVIRPDVAKWELSVKLHDEDHTVVASNNGPTFNVEVDGSKLNVTSTWNLASPLLSVNVDGTQRTVQCLSPDAGGNMSIQFLGTVYKVHILTKLAAELNKFMLEKVPKDTSSVLRSPKPGVVVAVSVKPGDMVAEGQEICVIEAMKMQNSMTAGKMGKVKLVHCKAGDTVGEGDLLVELE
uniprot
proteins
disease_involvement
DISEASE: Note=Propionic acidemia due to recessively inherited deficiency of PCCase activity often causes life-threatening ketosis and acidosis.
uniprot
proteins
ec_number
6.4.1.3
uniprot
proteins
function
FUNCTION: This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (By similarity). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA (By similarity). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity). {ECO:0000250|UniProtKB:P05165, ECO:0000250|UniProtKB:P0DTA4, ECO:0000250|UniProtKB:Q5LUF3}.
uniprot
proteins
mass_dalton
81623
uniprot
proteins
pathway_participation
PATHWAY: Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3. {ECO:0000250|UniProtKB:P05165}.
uniprot
proteins
post_translational_modification
PTM: Acetylated. {ECO:0000250|UniProtKB:Q91ZA3}.; PTM: The biotin cofactor is covalently attached to the C-terminal biotinyl-binding domain and is required for the catalytic activity. Biotinylation is catalyzed by HLCS. {ECO:0000250|UniProtKB:P05165}.
uniprot
proteins
pubmed
15060005
uniprot
proteins
pubmed
22673903
uniprot
proteins
pubmed
2745462
uniprot
proteins
sequence_length
737
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000250|UniProtKB:P05165}.
uniprot
proteins