Name: Anti-HADHA antibody
Price: 295.00 USD

Overview
- This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.
  
  Please contact us at for specific academic pricing.
Properties
- Host
  
  rabbit
  
  Antibody Type
  
  polyclonal
  
  Isotype
  
  IgG
  
  Reactivity
  
  human; mouse
  
  Immunogen
  
  Recombinant fusion protein containing a sequence corresponding to amino acids 484-763 of human HADHA (NP_000173. 2).
  
  Purification
  
  Affinity purification
  
  Conjugate
  
  Unconjugated
  
  Formulation
  
  PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  
  Storage
  
  Store at -20°C and avoid freeze-thaw cycles.
  
  Regulatory Status
  
  RUO
  
  Antigen
  
  Trifunctional Enzyme Subunit Alpha-Mitochondrial; 78 Kda Gastrin-Binding Protein; Monolysocardiolipin Acyltransferase; Tp-Alpha Includes-Long-Chain Enoyl-Coa Hydratase-Long Chain 3-Hydroxyacyl-Coa Dehydrogenase (Gene ID:3030; UniProt:P40939)
  
  * For research use only