After search, choose a molecule or a kind of categories listed in the left to narrow down your filter. If you have any problems, please contact us!

クイック注文

Text Size:AAA

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

データシートレビュー関連製品プロトコル
Human 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.
Human PHYH 抗体使用ガイド
特異度:Human PHYH
アプリケーション: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

保存: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
[クリックして画像を拡大]
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
[クリックして画像を拡大]
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
[クリックして画像を拡大]
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.

Human 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.
  • Product nameProduct name
    All information of our products is subject to change without notice. Please refer to COA enclosed in shipped package for the newest information.
    注意:すべての製品は、"研究目的のみに使用するものであり、診断または治療目的に使用するものではありません"