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FLASH GENE
Symbol HADHB contributors: mct - updated : 19-02-2018
HGNC name hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit
HGNC id 4803
Corresponding disease
MTPD mitochondrial trifunctional protein deficiency
Location 2p23.3      Physical location : 26.467.615 - 26.513.332
Synonym symbol(s) TFPB, MSTP029, ECHB, MTPB, TP-BETA
EC.number 2.3.1.16
DNA
TYPE functioning gene
STRUCTURE 45.72 kb     16 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked N status confirmed
Physical map
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
17 - 2279 - 452 - 1999 10075708
15 - 2151 - 459 - 1999 10075708
16 - 2196 - 474 - 1999 10075708
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart   highly
Digestiveliver    
Lymphoid/Immunethymus    
Urinarykidney    
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / hematopoieticbone marrow   
Connectivebone   
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
HOMOLOGY
Homologene
FAMILY
CATEGORY enzyme
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,organelle,mitochondria,inner
intracellular,cytoplasm,organelle,mitochondria,outer
intracellular,cytoplasm,organelle,mitochondria,matrix
intracellular,cytoplasm,organelle,membrane
intracellular,cytoplasm,organelle,endoplasmic reticulum
text ESR2 may be associated with HADHB in mitochondria
basic FUNCTION
  • fatty acid beta-oxidation trifunctional enzyme, beta subunit, harboring the long chain 3-oxoacyl-CoA thiolase, catalyzing three steps in the beta-oxidation of fatty acids, including the long-chain 3-hydroxyl-CoA dehydrogenase step
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism lipid/lipoprotein
    signaling
    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
  • interacting with ELAVL1, REN, PCBP1
  • ESR1 interacted with mitochondrial protein HADHB and affected the thiolytic cleavage activity of HADHB in beta-oxidation
  • ESR2 plays an inhibitory role in HADHB enzyme activity in the breast cancer cells
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MTPD
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS