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FLASH GENE
Symbol PCCA contributors: npt/ - updated : 10-07-2006
HGNC name propionyl Coenzyme A carboxylase, alpha polypeptide
HGNC id 8653
Corresponding disease
PCCA hyperglycinemia, ketotic, propionic acidemia A
Location 13q32.3      Physical location : 100.741.268 - 101.182.690
EC.number 6.4.1.3
DNA
TYPE functioning gene
STRUCTURE 441.35 kb     24 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked Y status confirmed
Map see COL4A1
Physical map
RAP2A 13q34 RAP2A, member of RAS oncogene family LOC121906 13q32.2 similar to Proteasome subunit alpha type 6 (Proteasome iota chain) (Macropain iota chain) (Multicatalytic endopeptidase complex iota chain) (27 kDa prosomal protein) (PROS-27) (p27K) LOC387943 13 LOC387943 KPNB3 13q32.2 karyopherin (importin) beta 3 FARP1 13q32.2-q32.3 FERM, RhoGEF (ARHGEF) and pleckstrin domain protein 1 (chondrocyte-derived) ZNF183L1 13q zinc finger protein 183-like 1 STK24 13q32 serine/threonine kinase 24 (STE20 homolog, yeast) HCP35 13q32.3 cytochrome c, somatic pseudogene SLC15A1 13q33-q34 solute carrier family 15 (oligopeptide transporter), member 1 DOCK9 13q32.1 dedicator of cytokinesis 9 LOC390421 13 similar to Glyceraldehyde 3-phosphate dehydrogenase, liver (GAPDH) PHGDHL1 13 phosphoglycerate dehydrogenase like 1 GPR18 13q32 G protein-coupled receptor 18 EBI2 13q32.3 Epstein-Barr virus induced gene 2 (lymphocyte-specific G protein-coupled receptor) LOC390422 13 similar to Epstein-Barr virus induced gene 2 TM9SF2 13q32.2 transmembrane 9 superfamily member 2 CLYBL 13q32.3 citrate lyase beta like ZIC5 13q32.2 Zic family member 5 (odd-paired homolog, Drosophila) ZIC2 13q32 Zic family member 2 (odd-paired homolog, Drosophila) PCCA 13q32 propionyl Coenzyme A carboxylase, alpha polypeptide LOC87769 13q32.3 hypothetical protein BC004360 FLJ14624 13q32.3 hypothetical protein FLJ14624 LOC390423 13 similar to ADP-ribosylation factor 4 VGCNL1 13q32.3 voltage gated channel like 1 ITGBL1 13q33 integrin, beta-like 1 (with EGF-like repeat domains) FGF14 13q34 fibroblast growth factor 14 TPP2 13q32-q33 tripeptidyl peptidase II LOC196541 13q33.1 similar to RIKEN cDNA 2310038H17 FLJ40176 13q33.1 hypothetical protein FLJ40176 LOC93081 13q33.1 hypothetical protein BC015148 KDELC1 13q33 KDEL (Lys-Asp-Glu-Leu) containing 1 BIVM 13q32-q33.1 basic, immunoglobulin-like variable motif containing
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
24 - 2518 - 728 - Campeau (1999)
23 - 2440 - 702 - Campeau (1999)
EXPRESSION
Type ubiquitous
constitutive of
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularvesselaorta   
Digestiveintestinelarge intestinecolon highly
 liver    
Endocrineadrenal gland   highly
Nervousbrain    
Reproductivefemale systembreastmammary gland highly
 male systemprostate   
Respiratorylung    
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectiveadipose  highly
cell lineage
cell lines
fluid/secretion
at STAGE
physiological period fetal
Text eye
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a C-terminal biotin-binding domain
  • a biotin carboxylase domain, defined by homology with other biotin-dependent carboxylases
  • an ATP-GRASP domain
  • mono polymer heteromer , heptamer
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    basic FUNCTION
  • propionyl-CoA carboxylase, alpha polypeptide, biotin-dependent
  • mitochondrial conversion of propionyl CoA to methylmalonyl CoA, branched chain
  • involved in valine, isoleucine catabolism,energy pathway
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits
  • INTERACTION
    DNA
    RNA
    small molecule cofactor,
  • biotin
  • protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) PCCA
    related resource Propionyl CoA Carboxylase Page
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS