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Product Name
Anti-PHYH antibody
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Description
Rabbit polyclonal to PHYH
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Tested applications
ELISA, IHC-P
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Species reactivity
Human PHYH
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Alternative names
AI256161 antibody; AI265699 antibody; LN1 antibody; Lnap1 antibody; LNAP1 antibody; lupus nephritis-associated peptide 1 antibody; PAHX antibody; PAHX antibody; peroxisomal antibody; peroxisomal antibody; PHYH1 antibody; phytanic acid oxidase antibody; phytanic acid oxidase antibody; phytanoil-CoA alpha hydroxylase antibody; phytanoyl-CoA 2 oxoglutarate dioxygenase antibody; phytanoyl-CoA alpha-hydroxylase antibody; phytanoyl-CoA alpha-hydroxylase antibody; phytanoyl-CoA dioxygenase antibody; phytanoyl-CoA dioxygenase antibody; RD antibody; RP11-24J20.1 antibody; RP23-349P20.1 antibody; LN1 antibody; PAHX antibody; LNAP1 antibody; PHYH1 antibody; PAHX antibody; Lnap1 antibody; AI256161 antibody; AI265699 antibody; RD antibody
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Isotype
Rabbit IgG
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Preparation
Produced in rabbits immunized with purified, recombinant Human PHYH (rh PHYH; O14832; Ser31-Leu338). PHYH specific IgG was purified by Human PHYH affinity chromatography.
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Clonality
Polyclonal
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Formulation
0.2 μm filtered solution in PBS with 5% trehalose
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Storage instructions
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free.
Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles. -
Applications
ELISA: 0.1-0.2 μg/mL
This antibody can be used at 0.1-0.2 μg/mL with the appropriate secondary reagents to detect Human PHYH. The detection limit for Human PHYH is approximately 0.00975 ng/well.
IHC-P: 0.1-2 μg/mL
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Background
PHYH belongs to the family of iron(II)-dependent oxygenases, which typically incorporate one atom of dioxygen into the substrate and one atom into the succinate carboxylate group. PHYH is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. It converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Defects in PHYH can cause Refsum disease (RD). RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues.
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References
- Mihalik SJ, et al. (1997) Identification of PAHX, a Refsum disease gene. Nat Genet. 17(2): 185-9.
- McDonough MA, et al. (2005) Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease. J Biol Chem. 280(49):41101-10.
- Jansen GA, et al. (1998) Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders. Clin Chim Acta. 271 (2):203-11.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"