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PHYH 抗體, 兔多抗, 抗原親和純化

  • PHYH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
  • PHYH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
  • PHYH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
產品資料評論相關產品實驗方法
 PHYH 抗體產品資訊
免疫原:Recombinant Human PHYH protein (Catalog#13368-HNAE)
Clone ID:
Ig類型:Rabbit IgG
濃度:
內毒素:
緩衝液:0.2 μm filtered solution in PBS with 5% trehalose
抗體製備方法: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.
 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
[點擊查看大圖]
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
[點擊查看大圖]
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 研究背景

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.

 PHYH 參考資料
  • 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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    貨號: 13368-RP02-50
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    Datasheet & Documentation

    All information of our products is subject to change without notice. Please refer to COA enclosed in shipped package for the newest information.
    請注意:所有產品都是“僅用於科研,而不能用於診斷或治療用途”