BackgroundThis 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.
DescriptionHADHA Monoclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide, pH 7.3.
SpecificityHuman, Mouse, Rat
IsotypeIgG
Uniprot IDP40939
PurificationAffinity Purified
ImmunogenRecombinant protein (or fragment). Please contact EpigenTek for more information.
StorageStore at -20°C. Avoid freeze / thaw cycles.
Alternative NamesGBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA; HA
ApplicationWB, IF/ICC, ELISA; Recommended dilution: WB, 1:1000 - 1:5000; IF, 1:200 - 1:800; ELISA, Recommended starting concentration is 1 µg/mL. Please optimize the concentration based on your specific assay requirements.
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