PHYH polyclonal Antibody | BS8115

PHYH polyclonal Antibody | BS8115 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse

Application: WB IF

Application Range: WB 1:500 - 1:2000 IF 1:10 - 1:100

Background: PHYH (phytanoyl-CoA 2-hydroxylase), also known as RD, LN1, PAHX or LNAP1, is a 338 amino acid protein that localizes to the peroxisome and plays an important role in fatty acid metabolism. Expressed in kidney, liver and T cells, PHYH uses iron and ascorbate as cofactors to catalyze the conversion of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA, a reaction that is involved in the alpha-oxidation of 3-methyl branched fatty acids. Defects in the gene encoding PHYH are associated with Refsum disease (RD), an autosomal recessive disorder that is characterized by retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment, all of which usually develop during the second or third decade of life.

Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: PHYH polyclonal Antibody detects endogenous levels of PHYH protein.

Molecular Weight: ~ 39 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: Phytanoyl-CoA dioxygenase, peroxisomal; Phytanic acid oxidase; Phytanoyl-CoA alpha-hydroxylase; PhyH; PHYH; PAHX

Immunogen: Recombinant full length Human PHYH.

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Pathway: