1.0 2018-05-07 22:21:32 UTC 2020-05-20 21:08:44 UTC BMDBP00253 Enzyme PCCB Involved in ATP binding 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). Metabolic intermediate metabolism Propanoate Metabolism SMP0087248 Propanoyl-CoA degradation Succinyl-CoA from propanoyl-CoA: step 1/3 Threonine and 2-Oxobutanoate Degradation SMP0087262 Valine, Leucine, and Isoleucine Degradation SMP0087234 BMDB0001275 Propionyl-CoA BMDB0001341 ADP BMDB0002310 S-Methylmalonyl-CoA BMDB0001493 3-Methylcrotonyl-CoA BMDB0000538 Adenosine triphosphate BMDB0000595 Hydrogen carbonate BMDB0001057 3-Methylglutaconyl-CoA BMDB0000973 Hydrogen phosphate BMDB0001362 Hydrogen 1 1q31-q36 >1620 bp ATGGCTGCAGCAATGCGCGTGGCTGTGGCCGGAGCCAGGCTCAGTGTTGTACTGAGAAGT CTCCGGGGCGCGGGGCGCAGCTTGTGCACCCAGCCTGTATCGGTTAACGAACGCATCGAG AACAAGCGGCAAGCTGCACTGCTGGGAGGAGGGCAGCGCCGCATTGACGCGCAGCATAAG CGAGGAAAGCTGACAGCCAGAGAGCGGATCAGTCTCTTGCTGGACCCTGGCAGTTTTGTT GAGAGCGACATGTTTGTGGAACACAGATGTGCAGATTTTGGAATGGCTGCTGATAAGAAT AAGTTCCCTGGAGACAGTGTGGTCACTGGACGAGGACGAATCAATGGAAGACTGGTTTAT GTCTTCAGTCAGGATTTTACAGTTTTTGGAGGTAGCCTGTCTGGAGCACATGCCCAAAAG ATCTGCAAAATCATGGACCAAGCCCTCACAGTGGGGGCTCCAGTGATTGGACTGAATGAC TCTGGAGGTGCACGGATTCAAGAGGGAGTGGAATCTTTGGCTGGCTATGCAGACATCTTT CTGAGGAATGTCACGGCATCCGGAGTCATCCCTCAGATTTCTCTGATCATGGGCCCATGT GCTGGTGGGGCTGTCTACTCCCCAGCCTTAACAGACTTCACGTTCATGGTGAAGGACACC TCCTACTTATTCATCACTGGCCCTGACGTTGTGAAGTCTGTCACCAATGAGGACGTTACT CAGGAGGAACTCGGGGGTGCCAGGACCCACACCACCATGTCAGGTGTGGCCCACAGGGCT TTTGAGAATGACGTTGATGCCTTGTGTAATCTCCGGGAATTCTTCAACTACCTGCCTCTC AGCAATCAGGACCCAGCTCCTGTCCTTGAGTGCCATGACCCCAGTGACCGTCTGGTTCCT GAGCTTGACACAATTGTCCCTTTGGAATCTACCAAAGCCTACAACATGGTGGACATCATA CATGCTGTTGTGGATGAGCGAGAATTTTTTGAGATCATGCCCAATTATGCCAAGAACATC ATTGTTGGTTTTGCAAGAATGAACGGGAGGACTGTTGGAATTGTTGGCAACCAACCTAAG GTGGCTTCAGGCTGCTTGGACATTAATTCTTCTGTGAAAGGGGCTCGTTTCGTCCGATTC TGTGACTCGTTCAACATTCCCCTCATCACTTTTGTGGATGTCCCCGGCTTCCTGCCTGGC ACCGCGCAGGAGTATGGGGGCATCATCCGCCACGGCGCCAAGCTGCTGTACGCGTTTGCA GAGGCCACTGTGCCCAAAGTGACGGTCATCACCAGGAAGGCCTACGGTGGTGCCTATGAT GTCATGAGCTCCAAGCACCTCTGTGGTGACACCAACTACGCCTGGCCCACGGCAGAGATT GCAGTCATGGGAGCAAAGGGTGCTGTGGAGATCATCTTCAAAGGGCACGAGAACGTGGAG GCTGCTCAGGCAGAGTACATCGAGAAGTTTGCCAACCCTTTTCCTGCAGCCGTGAGAGGG TTTGTGGACGACATCATCCAGCCTTCTTCCACACGTGCCCGAATCTGCTGTGACCTGGAT GTCCTGGCCAGCAAGAAGGTACAGCGCCCTTGGAGGAAACATGCGAATATTCCATTGTAA 539 58311.0 7.48 None >Propionyl-CoA carboxylase beta chain, mitochondrial MAAAMRVAVAGARLSVVLRSLRGAGRSLCTQPVSVNERIENKRQAALLGGGQRRIDAQHK RGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVY VFSQDFTVFGGSLSGAHAQKICKIMDQALTVGAPVIGLNDSGGARIQEGVESLAGYADIF LRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVT QEELGGARTHTTMSGVAHRAFENDVDALCNLREFFNYLPLSNQDPAPVLECHDPSDRLVP ELDTIVPLESTKAYNMVDIIHAVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPK VASGCLDINSSVKGARFVRFCDSFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFA EATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVE AAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL AAI09785.1 Q2TBR0 PCCB_BOVIN BC109784 PCCB