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PHYH 抗體, 兔多抗, 抗原親和純化

產品資料評論相關產品實驗方法
Human 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.
Human PHYH 抗體使用指導
特異性:Human PHYH
推薦應用:ELISA, IHC-P

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

儲存方法: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.
Human PHYH Antibody IHC Application Image
PHYH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
[點擊查看大圖]
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.

Human 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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