Recombinant Human PHYH-蛋白质/抗原/多肽-试剂-生物在线
Recombinant Human PHYH

Recombinant Human PHYH

商家询价

产品名称: Recombinant Human PHYH

英文名称: Recombinant Human PHYH

产品编号: PHYH-8581H

产品价格: 0

产品产地: USA

品牌商标: Creative Biomart

更新时间: null

使用范围: null

北京安必奇生物科技有限公司
  • 联系人 :
  • 地址 : 北京市通州区经海五路1号院22号楼
  • 邮编 :
  • 所在区域 : 北京
  • 电话 : 158****7462
  • 传真 :
  • 邮箱 : info@abace-biology.com

 http://www.creativebiomart.net/description_382721_12.htm

PHYH is a member of 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. It is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. PHYH has three substrates which are known as phytanoyl-CoA, 2-oxoglutarate, and O2, while its three products are 2-hydroxyphytanoyl-CoA, succinate, and CO2. PHYH 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.