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.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of PHYH. Species Crossreactivity: Human
