ECHA Rabbit Polyclonal Antibody

Ab type

Primary antibody

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.

Reactivity

Human, Mouse, Rat

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Immunogen

Synthesized peptide derived from human ECHA

Storage Stability

-20°C for 1 year

Concentration

1 mg/ml

MolecularWeight_Da

synonyms

ECHA; Trifunctional enzyme subunit alpha; mitochondrial; 78 kDa gastrin-binding protein; TP-alpha [Includes: Long-chain enoyl-CoA hydratase; EC 4.2.1.17; Long chain 3-hydroxyacyl-CoA dehydrogenase; EC 1.1.1.211;