RM2879

HADHA (17C5) Rabbit Monoclonal Antibody

GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA

常规抗体

WB

Human,Mouse,Rat

Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% New type preservative N and 0.05% BSA.

A synthetic peptide of human HADHA

WB: 1:1000 ICC/IF: 1:50 FC: 1:20 IP: 1:20

78kDa

83kDa

-20°C/1 year

Rabbit

IgG

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]

Affinity Purification