BS8115

antibody from Bioworld Technology, Inc
Targeting: PHYH PAHX, PHYH1, RD
Provider product page for BS8115
Western blot
Immunohistochemistry

Antibody data

Product number
BS8115 - Provider product page
Provider
Bioworld Technology, Inc
Product name
PHYH polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human PHYH.
Description
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.
Reactivity
Human
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
PHYH protein structure - BS8115 shown in red.