PHYH 蛋白
Quick Overview for PHYH 蛋白 (ABIN7317253)
抗原
See all PHYH 蛋白蛋白类型
宿主
资源
纯度
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原理
- Recombinant Human PHYH Protein
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序列
- Ser31-Leu338
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产品特性
- A DNA sequence encoding the human PHYH (O14832) (Ser31-Leu338) was expressed, with a N-terminal Met.
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Phytanoyl-CoA 2-Hydroxylase (PHYH) (AA 1-338) protein (GST tag)
PHYH 宿主: 人 宿主: 小麦胚 Recombinant ELISA, WB, AP, AA
Phytanoyl-CoA 2-Hydroxylase (PHYH) (Transcript Variant 1) protein (Myc-DYKDDDDK Tag)PHYH 宿主: 人 宿主: HEK-293 Cells Recombinant > 80 % as determined by SDS-PAGE and Coomassie blue staining AbP, STD
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Please refer to the printed manual for detailed information.
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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
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储存条件
- 4 °C,-20 °C,-80 °C
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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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- PHYH (Phytanoyl-CoA 2-Hydroxylase (PHYH))
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别名
- PHYH
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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
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分子量
- 35.6 kDa
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UniProt
- O14832
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途径
- Monocarboxylic Acid Catabolic Process
抗原
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