PHS, Human, 重组蛋白 (His) TMPJ-01043

Product Introduction
Bioactivity


英文名: PHS Protein, Human, Recombinant (His)

描述: Pterin-4-α-Carbinolamine Dehydratase (PCBD1) is the founding member of the Pterin-4-α-Carbinolamine Dehydratase Family. PCBD1 is involved in Tetrahydrobiopterin biosynthesis. It seems to prevent the formation of 7-Pterins and accelerate the formation of Quinonoid-BH2. Furthermore, PCBD1 regulates the homodimerization of the transcription factor Hepatocyte Nuclear Factor 1 (HNF1) and enhances its transcriptional activity. Defects in PCBD1 are the cause of BH4-Deficient Hyperphenylalaninemia Type D (HPABH4D). HPABH4D is characterized by the excretion of 7-substituted Pterins in the urine of affected patients.

存储条件: store at low temperatureLyophilized powder: -20~-80°C for 1 year | Solution: -80°C for 6~12 month

关键字: 4-a-Hydroxy-Tetrahydropterin Dehydratase | Pterin-4-a-Carbinolamine Dehydratase

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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