PHYH 蛋白
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- 抗原 See all PHYH 蛋白
- PHYH (Phytanoyl-CoA 2-Hydroxylase (PHYH))
- 蛋白类型
- Recombinant
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宿主
- 人
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资源
- 大肠杆菌(E. Coli)
- 原理
- Recombinant Human PHYH Protein
- 序列
- Ser31-Leu338
- 产品特性
- A DNA sequence encoding the human PHYH (O14832) (Ser31-Leu338) was expressed, with a N-terminal Met.
- 纯度
- > 80 % as determined by reducing SDS-PAGE.
- Top Product
- Discover our top product PHYH 蛋白
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- 限制
- 仅限研究用
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- 状态
- Lyophilized
- 溶解方式
- Please refer to the printed manual for detailed information.
- 缓冲液
- Lyophilized from sterile 20 mM mops, 10 % glycerol, 2 mM DDT, 1 mM EDTA, 0.2 mM PMSF, 0.2M NaCl, pH 7.2
- 储存条件
- 4 °C,-20 °C,-80 °C
- 储存方法
- Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
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- 抗原
- PHYH (Phytanoyl-CoA 2-Hydroxylase (PHYH))
- 别名
- PHYH (PHYH 产品)
- 别名
- zgc:110203 Protein, LN1 Protein, LNAP1 Protein, PAHX Protein, PHYH1 Protein, RD Protein, AI256161 Protein, AI265699 Protein, Lnap1 Protein, phytanoyl-CoA 2-hydroxylase Protein, phytanoyl-CoA hydroxylase-like Protein, phytanoyl-CoA hydroxylase Protein, PHYH Protein, LOC478001 Protein, phyh Protein, Phyh Protein
- 背景
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Background: 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.
Synonym: LN1,LNAP1,PAHX,PHYH1,RD
- 分子量
- 35.6 kDa
- UniProt
- O14832
- 途径
- Monocarboxylic Acid Catabolic Process
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