PCCB Defects in PCCB are the cause of propionic acidemia type II (PA-2). PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC 6.4.1.3; Ligase; Mitochondrial
Chromosomal Location of Human Ortholog: 3q22.3
Cellular Component:  cytosol; mitochondrial matrix; mitochondrion
Molecular Function:  ATP binding; propionyl-CoA carboxylase activity; protein binding
Biological Process:  biotin metabolic process; short-chain fatty acid catabolic process
Disease: Propionic Acidemia
Reference #:  P05166 (UniProtKB)
Alt. Names/Synonyms: DKFZp451E113; PCCase subunit beta; PCCB; Propanoyl-CoA:carbon dioxide ligase subunit beta; propionyl Coenzyme A carboxylase, beta polypeptide; Propionyl-CoA carboxylase beta chain, mitochondrial
Gene Symbols: PCCB
Molecular weight: 58,216 Da
Basal Isoelectric point: 7.56  Predict pI for various phosphorylation states
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PCCB

Protein Structure Not Found.

Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene