Names & Taxonomy
- Uniprot ID:
- P05166
- Entry Name:
- PCCB_HUMAN
- Status:
- reviewed
- Protein Names:
- Propionyl-CoA carboxylase beta chain, mitochondrial (PCCase subunit beta) (EC 6.4.1.3) (Propanoyl-CoA:carbon dioxide ligase subunit beta)
- Gene Names:
- PCCB
- Gene Names Primary:
- PCCB
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 539
- Sequence:
- MAAALRVAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVYVFSQDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIFLRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVTQEELGGAKTHTTMSGVAHRAFENDVDALCNLRDFFNYLPLSSQDPAPVRECHDPSDRLVPELDTIVPLESTKAYNMVDIIHSVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPKVASGCLDINSSVKGARFVRFCDAFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFAEATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVEAAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix
Function
- Pathway:
- Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3.
- Catalytic Activity:
- ATP + propanoyl-CoA + HCO(3)(-) = ADP + phosphate + (S)-methylmalonyl-CoA.
- Cross Reference Drug Bank:
- DB00121 DB00161
- Gene Ontology Go:
- cytosol
mitochondrial matrix
mitochondrion
ATP binding
propionyl-CoA carboxylase activity
biotin metabolic process
cellular lipid metabolic process
fatty acid beta-oxidation
short-chain fatty acid catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Biological Process:
- biotin metabolic process
cellular lipid metabolic process
fatty acid beta-oxidation
short-chain fatty acid catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Molecular Function:
- ATP binding
propionyl-CoA carboxylase activity - Gene Ontology Cellular Component:
- cytosol
mitochondrial matrix
mitochondrion - Keywords:
- ATP-binding
Acetylation
Alternative splicing
Complete proteome
Direct protein sequencing
Disease mutation
Ligase
Mitochondrion
Nucleotide-binding
Phosphoprotein
Polymorphism
Reference proteome
Transit peptide