Anti-PHYH Antibody

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Anti-PHYH Antibody (Rabbit Polyclonal antibody) General Information

Product name
Anti-PHYH Antibody
Validated applications
ELISA,IHC-P
Species reactivity
Reacts with: Human
Specificity
Human PHYH
Immunogen
Recombinant Human PHYH protein (Catalog#13368-HNAE)
Preparation
Produced in rabbits immunized with purified, recombinant Human PHYH (rh PHYH; Catalog#13368-HNAE; O14832; Ser31-Leu338). PHYH specific IgG was purified by Human PHYH affinity chromatography.
Source
Polyclonal Rabbit IgG
Purification
Protein A & Antigen Affinity
Formulation
0.2 μm filtered solution in PBS
Conjugate
Unconjugated
Form
Liquid
Shipping
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Storage
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. Avoid repeated freeze-thaw cycles.

Anti-PHYH Antibody (Rabbit Polyclonal antibody) Validated Applications

Application Dilution
ELISA 1:5000-1:10000
IHC-P 1:500-1:2000
Please Note: Optimal concentrations/dilutions should be determined by the end user.

Anti-PHYH Antibody (Rabbit Polyclonal antibody) Images

Immunochemical staining of human PHYH in human spleen with rabbit polyclonal antibody (1:1000, formalin-fixed paraffin embedded sections).
Immunochemical staining of human PHYH in human liver with rabbit polyclonal antibody (1:1000, formalin-fixed paraffin embedded sections).
Immunochemical staining of human PHYH in human kidney with rabbit polyclonal antibody (1:1000, formalin-fixed paraffin embedded sections).

Anti-PHYH Antibody: Alternative Names

Anti-LN1 Antibody; Anti-LNAP1 Antibody; Anti-PAHX Antibody; Anti-PHYH1 Antibody; Anti-RD Antibody

PHYH Background Information

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.
Full Name
phytanoyl-CoA 2-hydroxylase
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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