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PHYH 蛋白

PHYH 宿主: 人 宿主: 大肠杆菌(E. Coli) Recombinant > 80 % as determined by reducing SDS-PAGE.
产品编号 ABIN7317253
发货至: 中国
  • 抗原 See all PHYH 蛋白
    PHYH (Phytanoyl-CoA 2-Hydroxylase (PHYH))
    蛋白类型
    Recombinant
    宿主
    • 2
    • 1
    • 1
    资源
    • 2
    • 1
    • 1
    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
  • 限制
    仅限研究用
  • 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.
  • 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

    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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