Antibody data
- Antibody Data
- Antigen structure
- References [0]
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- Validations
- Western blot [1]
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- 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.
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Supportive validation
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- Bioworld Technology, Inc (provider)
- Main image
- Experimental details
- WesternBlot (WB) analysis of PHYH polyclonal antibody