Product Name :HADHA mouse monoclonal antibody (KU)
Reactivity :Human, Rat, Mouse
Application_all :WB 1:500
Background :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. [provided by RefSeq, Jul 2008]
Product :1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Purification&Purity :The antibody was affinity-purified from mouse ascites fluids or tissue culture supernatant by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Note :For research use only, not for use in diagnostic procedure.
Alternative Name :ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA
Immunogen :Human recombinant protein fragment corresponding to amino acids 106-325 of human HADHA (NP_000173) produced in E.coli.
Modification :Unmodification
