UniProt:P05166 PCCB

chain
  • transit peptide:1-28
  • chain:29-539
checksum A2DAAC00312D3C0F
comment
  • 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 (PubMed:15890657, PubMed:6765947). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:15890657, PubMed:6765947). 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 at a much lower rate (PubMed:6765947). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).CATALYTIC ACTIVITY propanoyl-CoA + hydrogencarbonate + ATP = (S)-methylmalonyl-CoA + ADP + phosphate + H(+)CATALYTIC ACTIVITY butanoyl-CoA + hydrogencarbonate + ATP = (2S)-ethylmalonyl-CoA + ADP + phosphate + H(+)BIOPHYSICOCHEMICAL PROPERTIES Optimum pH is 7.2-8.8 for the propionyl-CoA carboxylase activity as measured for the holoenzyme.PATHWAY Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3.SUBUNIT The holoenzyme is a dodecamer composed of 6 PCCA/alpha subunits and 6 PCCB/beta subunits.INTERACTION The beta subunit contains the carboxyl transferase (CT) domain.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the AccD/PCCB family.
crossReference
databaseName UniProt
dbId 61380
description
  • recommendedName: fullName evidence="25"Propionyl-CoA carboxylase beta chain, mitochondrial shortName: PCCase subunit beta ecNumber evidence="13 17"6.4.1.3 alternativeName: Propanoyl-CoA:carbon dioxide ligase subunit beta
displayName UniProt:P05166 PCCB
geneName
  • PCCB
identifier P05166
isSequenceChanged false
keyword
  • 3D-structure
  • Acetylation
  • Alternative splicing
  • ATP-binding
  • Direct protein sequencing
  • Disease variant
  • Ligase
  • Mitochondrion
  • Nucleotide-binding
  • Phosphoprotein
  • Proteomics identification
  • Reference proteome
  • Transit peptide
modified [InstanceEdit:9948485] Weiser, Joel, 2025-05-21
moleculeType Protein
name
  • PCCB
otherIdentifier
  • 11726043_a_at
  • 11755510_a_at
  • 16946016
  • 212694_PM_s_at
  • 212694_s_at
  • 2644015
  • 2644016
  • 2644017
  • 2644018
  • 2644019
  • 2644021
  • 2644022
  • 2644023
  • 2644024
  • 2644025
  • 2644026
  • 2644027
  • 2644028
  • 2644030
  • 2644031
  • 2644032
  • 2644035
  • 2644036
  • 2644040
  • 2644042
  • 2644043
  • 2644044
  • 2644045
  • 2644046
  • 2644047
  • 2644048
  • 2644049
  • 2644050
  • 2644051
  • 2644052
  • 2644053
  • 2644054
  • 2644055
  • 2644056
  • 2644057
  • 2644058
  • 2644060
  • 36561_at
  • 5096
  • 8082886
  • A_14_P100373
  • A_14_P102146
  • A_14_P115437
  • A_23_P121051
  • A_24_P302797
  • A_24_P302802
  • GE59983
  • GO:0000166
  • GO:0003989
  • GO:0004658
  • GO:0005515
  • GO:0005524
  • GO:0005739
  • GO:0005759
  • GO:0005829
  • GO:0006520
  • GO:0006629
  • GO:0006631
  • GO:0006633
  • GO:0009062
  • GO:0009081
  • GO:0009317
  • GO:0016740
  • GO:0016743
  • GO:0016874
  • GO:0019626
  • GO:1902494
  • HMNXSV003021554
  • HMNXSV003025490
  • Hs.63788.0.S1_3p_a_at
  • ILMN_1761010
  • M31169_s_at
  • PH_hs_0003983
  • PH_hs_0035441
  • S67325_at
  • TC03000733.hg
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • PCCB_HUMAN
  • B7Z2Z4
  • Q16813
  • Q96CX0
sequenceLength 539
species [Species:48887] Homo sapiens
stId uniprot:P05166
url http://purl.uniprot.org/uniprot/P05166
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