P07633
uniprot
P07633
canonical
amino_acid_sequence
MAAVIRIRAMAAGTRLRVLNCGLGTTIRSLCSQPVSVNERIENKRHAALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFLESDMFVEHRCADFGMAAEKNKFPGDSVVTGRGRINGRLVYVFSQDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIFLRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPEFVKSVTNEDVTQEQLGGAKTHTTVSGVAHRAFDNDVDALCNLREFLNFLPLSNQDPASIRECHDPSDRLVPELDTVVPLESSKAYNMLDIIHAVIDEREFFEIMPNYAKNIVIGFARMNGRTVGIVGNQPNVASGCLDINSSVKGARFVRFCDAFSIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFAEATVPKITVITRKAYGGAYDVMSSKHLLGDTNYAWPTAEIAVMGAKGAVEIIFKGHEDVEAAQAEYVEKFANPFPAAVRGFVDDIIQPSSTRARICCDLEVLASKKVHRPWRKHANVPL
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:P05166, ECO:0000250|UniProtKB:P79384, ECO:0000250|UniProtKB:Q168G2}.
uniprot
proteins
mass_dalton
58626
uniprot
proteins
pathway_participation
PATHWAY: Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3. {ECO:0000250|UniProtKB:P05166}.
uniprot
proteins
protein_family
AccD/PCCB family
uniprot
proteins
pubmed
3464942
uniprot
proteins
sequence_length
541
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000250|UniProtKB:P05166}.
uniprot
proteins