abx004088

antibody from Abbexa

Targeting: HADHA GBP, LCEH, LCHAD, MTPA

Western blot (Recommended by provider)

Immunocytochemistry (Recommended by provider)

Antibody Data

Product number

abx004088

Provider

Abbexa

Product name

Trifunctional Enzyme Subunit Alpha, Mitochondrial (HADHA) Antibody

Antibody type

Polyclonal

Description

HADHA Antibody is a Rabbit Polyclonal antibody against HADHA. 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

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

100 µl

Concentration

> 1 mg/ml

Storage

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.