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FLASH GENE
Symbol ACADVL contributors: - updated : 12-10-2006
HGNC name acyl-Coenzyme A dehydrogenase, very long chain
HGNC id 92
Corresponding disease
ACADVL ACADVL deficiency presenting a severe childhood form
Location 17p13.1      Physical location : 7.123.152 - 7.128.584
Synonym name very-long-chain specific acyl-CoA dehydrogenase, mitochondrial
Synonym symbol(s) LCACD, VLCAD, ACAD6
EC.number 1.13.99.13
DNA
TYPE functioning gene
SPECIAL FEATURE head to head
text head to head with DLG4
STRUCTURE 5.00 kb     20 Exon(s)
10 Kb 5' upstream gene genomic sequence study
regulatory sequence Binding site
text structure
  • AP-2 binding site regulating the promoter and a 15 bp DNA motif
  • bidirectonal promoter with DLG4
  • MAPPING cloned Y linked N status confirmed
    Physical map
    ALOX12P2 17p13.1 arachidonate 12-lipoxygenase pseudogene 2 ALOX12 17p13.1 arachidonate 12-lipoxygenase LOC388328 17 LOC388328 MGC49942 17p13.2 hypothetical protein MGC49942 BCL6B 17p13.1 B-cell CLL/lymphoma 6, member B (zinc finger protein) LOC201232 SLC16A11 17p13.2 solute carrier family 16 (monocarboxylic acid transporters), member 11 CLECSF13 2p13.3 likely ortholog of mouse C-type (calcium dependent, carbohydrate recognition domain) lectin, superfamily member 13 ASGR2 17p13.1 asialoglycoprotein receptor 2 ASGR1 17p12 asialoglycoprotein receptor 1 DLG4 17p13.1 discs, large (Drosophila) homolog 4 ACADVL 17p13.1 acyl-Coenzyme A dehydrogenase, very long chain DVL2 17p13.2 dishevelled, dsh homolog 2 (Drosophila) MGC2941 17p13.2 hypothetical protein MGC2941 GABARAP 17p13.1 GABA(A) receptor-associated protein DULLARD 17p13 dullard homolog (Xenopus laevis) DERP6 17p13.2 S-phase 2 protein CLDN7 17p13.1 claudin 7 SLC2A4 17p13.1 solute carrier family 2 (facilitated glucose transporter), member 4 YBX2 17p11.2-p13.1 germ cell specific Y-box binding protein EIF5A 17p13-p12 eukaryotic translation initiation factor 5A GPS2 17p13 G protein pathway suppressor 2 LOC390760 17 similar to RIKEN cDNA 2810408A11 CENTB1 17p13.2 centaurin, beta 1
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    - - 2320 70.4 655 - Zhang
    - - 2254 - 633 - Zhang
    EXPRESSION
    Type ubiquitous
    constitutive of
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart    
     vessel    
    Digestiveliver   highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Epithelialsensoryvisualphotoreceptor layer 
    Muscularstriatumcardiac  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    physiological period embryo
    Text developing kidney
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • an all-alpha all terminal domain, transmembrane segment
  • all-alpha, four helical up-and-down bundle
  • C terminal domain
  • mono polymer homomer , dimer
    isoforms Precursor
    HOMOLOGY
    interspecies ortholog to murine Acadvl
    Homologene
    FAMILY acyl-CoA dehydrogenase family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    basic FUNCTION
  • acyl-CoA dehydrogenase, very long chain 14 to 20C, first step of the fatty acid beta-oxidation
  • transferring an electron to ETF
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS electron transport
    PATHWAY
    metabolism energetic , lipid/lipoprotein
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule cofactor, nucleotide,
    FAD+
    protein
    cell & other
    REGULATION
    activated by activator protein 2 (TAFP2A)
    ASSOCIATED DISORDERS
    corresponding disease(s) ACADVL
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS