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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
Corresponding disease
LCHAD long chain hydroxyacyl-CoA dehydrogenase deficiency
Location 2p23.3      Physical location : 26.413.504 - 26.467.594
Synonym name
  • 78 kDa gastrin-binding protein
  • 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit
  • 3-oxoacyl-CoA thiolase
  • mitochondrial trifunctional enzyme, alpha subunit
  • Synonym symbol(s) TFPA, MTPA, GBP, GBP, HADH, MGC1728, TP-ALPHA
    EC.number 4.2.1.17, 1.1.1.35
    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
    Physical map
    POMC 2p23.3 proopiomelanocortin (adrenocorticotropin/ beta-lipotropin/ alpha-melanocyte stimulating hormone/ beta-melanocyte stimulating hormone/ beta-endorphin) DNMT3A 2p23 DNA (cytosine-5-)-methyltransferase 3 alpha DTNB 2p23-p22 dystrobrevin, beta ASXL2 2p24.1 additional sex combs like 2 (Drosophila) KIF3C 2p23 kinesin family member 3C LOC391357 2 similar to Ubiquinol-cytochrome C reductase complex 11 kDa protein, mitochondrial precursor (Mitochondrial hinge protein) (Cytochrome C1, nonheme 11 kDa protein) (Complex III subunit VIII) LOC391358 2 similar to hypothetical protein HSPC152 RAB10 2p24.1 RAB10, member RAS oncogene family LOC391359 2 similar to Epithelial membrane protein-2 (EMP-2) (XMP protein) LOC130482 2p24.1 similar to SWI/SNF-related matrix-associated actin-dependent regulator of chromatin e1; mammalian chromatin remodeling complex BRG1-associated factor 57 PPIL1P1 2p23.3 peptidylprolyl isomerase (cyclophilin)-like 1 pseudogene 1 LOC150946 2p24.1 hypothetical protein LOC150946 HADHA 2p23 hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit HADHB 2p23 hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit GPR113 2p24.1 G protein-coupled receptor 113 KIAA1724 2p24.1 selenoprotein I, 1 MGC16372 2p24.1 CG10958-like OTOF 2p23-p22 otoferlin LOC388936 2 similar to RIKEN cDNA 1700001C02 LOC130106 2p24.1 similar to Calcium and integrin-binding protein 1 (Calmyrin) (DNA-PKcs interacting protein) (Kinase interacting protein) (KIP) (CIB) KCNK3 2p24.1-p23.1 potassium channel, subfamily K, member 3 FLJ20555 CENPA 2p23.1-p22.3 centromere protein A, 17kDa DPYSL5 2p23-p21 dihydropyrimidinase-like 5 MAPRE3 2p23.3-p23.1 microtubule-associated protein, RP/EB family, member 3 FLJ20254 2p23.3 hypothetical protein FLJ20254 FLJ21839 2p23.3 hypothetical protein FLJ21839 EMILIN1 2p23.3-p23.2 elastin microfibril interfacer 1 KHK 2p23.3-p23.2 ketohexokinase (fructokinase) CGREF1 2p23.3 cell growth regulator with EF hand domain 1 ABHD1 2p23.3 abhydrolase domain containing 1 PREB 2p23 prolactin regulatory element binding MGC44505 2p23.3 hypothetical protein MGC44505 LOC391360 2 similar to class II basic helix-loop-helix protein TCF23
    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