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PHYH 抗体, ウサギポリクローナル抗体, Antigen Affinity Purified

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人々 PHYH 抗体製品情報
免疫原:Recombinant Human PHYH protein (Catalog#13368-HNAE)
Clone ID:
Ig タイプ:Rabbit IgG
濃度:
エンドトキシン:
製剤:0.2 μm filtered solution in PBS with 5% trehalose
調製: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.
人々 PHYH Antibody IHC Application Image
PHYH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
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Caption:
Immunochemical staining of human PHYH in human kidney with rabbit polyclonal antibody (1 µg/mL, formalin-fixed paraffin embedded sections).
PHYH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
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Caption:
Immunochemical staining of human PHYH in human liver with rabbit polyclonal antibody (1 µg/mL, formalin-fixed paraffin embedded sections).
PHYH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
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Caption:
Immunochemical staining of human PHYH in human spleen with rabbit polyclonal antibody (1 µg/mL, formalin-fixed paraffin embedded sections).
Other PHYH Antibody Products
PHYH 背景

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.

人々 PHYH 参考文献
  • 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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    カタログ: 13368-RP02-50
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