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FLASH GENE
Symbol GCDH contributors: mct/npt - updated : 10/12/2008
HGNC name glutaryl-Coenzyme A dehydrogenase
HGNC id 4189
Corresponding disease
GA1 glutaric acidemia 1
Location 19p13.2      Physical location : 13.001.973 - 13.010.782
Synonym name glutaryl-CoA dehydrogenase, mitochondrial
Synonym symbol(s) ACAD5, GCD
EC.number 1.3.99.7
DNA
TYPE functioning gene
STRUCTURE 8.81 kb     11 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
12 - 1839 48.1 438 - Fu, Rao (2006)
12 - 1607 - 428 - Fu, Rao (2006)
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveintestinesmall intestine  highly
 stomach   highly
Lymphoid/Immunespleen   highly
 thymus   highly
 tonsils   highly
Reproductivefemale systemovary  highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / hematopoieticbone marrow   
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • acyl-CoA dehydrogenase, C-terminal domain
  • conjugated FlavoP
    mono polymer homomer , tetramer
    HOMOLOGY
    Homologene
    FAMILY
  • acyl-CoA dehydrogenase family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    text
  • after import into mitochondria 44 N-terminal amino acid residues are cleaved off (Keyser 2008)
  • basic FUNCTION
  • glutaryl-CoA dehydrogenase, flavoprotein dependent, lysine oxidation
  • catalyzing the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule cofactor,
    FAD
    protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) GA1
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS