Identification
BMDB Protein ID BMDBP00253
Secondary Accession Numbers None
Name Propionyl-CoA carboxylase beta chain, mitochondrial
Synonyms Not Available
Gene Name PCCB
Protein Type Enzyme
Biological Properties
General Function Involved in ATP binding
Specific 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).
Pathways
  • Metabolic intermediate metabolism
  • Propanoate Metabolism
  • Propanoyl-CoA degradation
  • Succinyl-CoA from propanoyl-CoA: step 1/3
  • Threonine and 2-Oxobutanoate Degradation
  • Valine, Leucine, and Isoleucine Degradation
Reactions
3-Methylcrotonyl-CoA + Adenosine triphosphate + Hydrogen carbonate → 3-Methylglutaconyl-CoA + ADP details
Propionyl-CoA + Adenosine triphosphate + Hydrogen carbonate → S-Methylmalonyl-CoA + ADP + Hydrogen phosphate details
Propionyl-CoA + Hydrogen carbonate + Adenosine triphosphate → S-Methylmalonyl-CoA + ADP + Hydrogen details
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 1
Locus 1q31-q36
SNPs PCCB
Gene Sequence
>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
Protein Properties
Number of Residues 539
Molecular Weight 58311.0
Theoretical pI 7.48
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions Not Available
Protein Sequence
>Propionyl-CoA carboxylase beta chain, mitochondrial
MAAAMRVAVAGARLSVVLRSLRGAGRSLCTQPVSVNERIENKRQAALLGGGQRRIDAQHK
RGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVY
VFSQDFTVFGGSLSGAHAQKICKIMDQALTVGAPVIGLNDSGGARIQEGVESLAGYADIF
LRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVT
QEELGGARTHTTMSGVAHRAFENDVDALCNLREFFNYLPLSNQDPAPVLECHDPSDRLVP
ELDTIVPLESTKAYNMVDIIHAVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPK
VASGCLDINSSVKGARFVRFCDSFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFA
EATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVE
AAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL
GenBank ID Protein AAI09785.1
UniProtKB/Swiss-Prot ID Q2TBR0
UniProtKB/Swiss-Prot Entry Name PCCB_BOVIN
PDB IDs Not Available
GenBank Gene ID BC109784
GeneCard ID PCCB
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available