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FLASH GENE
Symbol HADHA contributors: mct/npt - updated : 07-02-2018
HGNC name hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
HGNC id 4801
DNA
TYPE functioning gene
SPECIAL FEATURE
text linked to HADHB in head to head arrangement on opposite strand with a common 350 bp flanking region
STRUCTURE 54.10 kb     20 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
20 - 3048 59 763 - Taylor (2009)
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Nervousspinal cordanterior horn   
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Epithelialsensoryvisualphotoreceptor layer 
Muscularstriatumcardiacmyocardium 
cells
SystemCellPubmedSpeciesStageRna symbol
Respiratoryepithelial cell
cell lineage
cell lines
fluid/secretion
at STAGE
physiological period embryo
Text developing brain and neural retina, metanephros, gonads, gut
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a subunit hydratase domain
  • linker domain
  • dehydrogenase domain
  • mono polymer heteromer , hexamer
    HOMOLOGY
    Homologene
    FAMILY
  • enoyl-CoA hydratase/isomerase family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,organelle,membrane
    basic FUNCTION
  • fatty acid beta-oxidation trifunctional enzyme, alpha subunit harboring the long chain 2-enoyl-CoA hydratase and the long chain 3-hydroxyacyl-CoA dehydrogenase domains, beta subunit harboring the long chain ICoA thiolase activity
  • specific for the resynthesis of Cardiolipin from HADHA and acyl coenzyme A (Taylor 2009)
  • mitochondrial monolysocardiolipin acyltransferase involved in the remodeling of cardiolipin (Taylor 2009)
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism lipid/lipoprotein
    signaling
  • fatty acid beta-oxidation cycle, step 2
  • fatty acid beta-oxidation cycle, step 3
  • a component
  • Mitochondrial trifunctional protein (MTP) is a hetero-octamer composed of four alpha- and four beta-subunits (HADHA, HADHB) that catalyzes the final three steps of mitochondrial beta-oxidation of long chain fatty acids
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • gastrin binding
  • cell & other
    REGULATION
    Other replication dependent
    ASSOCIATED DISORDERS
    corresponding disease(s) LCHAD
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target might serve as a potential therapeutic target in BTHS patients (Taylor 2009)
    ANIMAL & CELL MODELS