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FLASH GENE
Symbol PCCB contributors: mct - updated : 29-04-2013
HGNC name propionyl Coenzyme A carboxylase, beta polypeptide
HGNC id 8654
Corresponding disease
PCCB hyperglycinemia, ketotic, propionic acidemia B
Location 3q22.3      Physical location : 135.969.166 - 136.049.015
Synonym name
  • PCCase subunit beta
  • propanoyl-CoA:carbon dioxide ligase subunit beta
  • Synonym symbol(s) DKFZp451E113
    EC.number 6.4.1.3
    DNA
    TYPE functioning gene
    STRUCTURE 79.85 kb     15 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    15 - 1825 58.1 539 - 1998 9683601
    16 - 1885 - 559 - 1998 9683601
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    blood / hematopoieticspleen   highly
    Digestiveliver   highly
     stomach    
    Nervousbrain   highly
    Reproductivemale systemprostate   
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / Hematopoieticbone marrow   
    Lymphoid    
    cell lineage
    cell lines schwannoma tumor cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a carboxyltransferase domain
  • secondary structure six alpha and six beta subunit
    mono polymer heteromer , polymer
    HOMOLOGY
    interspecies homolog to rattus Pccb
    Homologene
    FAMILY
  • accd/pccb family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    basic FUNCTION
  • propionyl-CoA carboxylase, beta polypeptide, biotin-dependent
  • mitochondrial conversion of propionyl CoA to methylmalonyl CoA, branched chain
  • valine, isoleucine catabolism, energy pathway
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism aminoacid
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) PCCB
    related resource Propionyl CoA Carboxylase Page
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS