PHYH, Human, 重组蛋白 TMPY-03109
品牌 | 厂商性质 | 产地 | 货期 |
---|---|---|---|
TargetMol | 生产商 | 美国 | 现货 |
性能特点
生化试剂,可用于动物细胞实验
规格 | 价格 | 操作 |
---|---|---|
100 μg | ¥4,460.00 | 询底价 |
Product Introduction
Bioactivity
英文名: PHYH Protein, Human, Recombinant
描述: 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.
存储条件: Lyophilized powder: -20~-80°C for 1 year | Solution: -80°C for 6~12 month
关键字: LN 1 | PHYH 1 | LN-1 | LNAP-1 | LNAP 1 | PHYH-1
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注:该产品未在中华人民共和国食品药品监督管理部门申请医疗器械注册和备案,不可用于临床诊断或治疗等相关用途