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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
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