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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.

| 规格 | 价格 | 库存 | 数量 |
|---|---|---|---|
| 5 μg | ¥ 693 | 6-8日内发货 | |
| 10 μg | ¥ 1,170 | 6-8日内发货 | |
| 20 μg | ¥ 1,860 | 5日内发货 | |
| 50 μg | ¥ 3,470 | 5日内发货 | |
| 100 μg | ¥ 4,930 | 5日内发货 | |
| 200 μg | ¥ 7,350 | 5日内发货 | |
| 500 μg | ¥ 12,100 | 5日内发货 | |
| 1 mg | ¥ 17,300 | 5日内发货 |
| 生物活性 | Activity has not been tested. It is theoretically active, but we cannot guarantee it. If you require protein activity, we recommend choosing the eukaryotic expression version first. |
| 产品描述 | 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. |
| 种属 | Human |
| 表达系统 | E. coli |
| 标签 | N-6xHis |
| 蛋白编号 | P61457 |
| 别名 | Pterin-4-α-Carbinolamine Dehydratase,Pterin-4-Alpha-Carbinolamine Dehydratase,Pterin Carbinolami,PHS,Phenylalanine Hydroxylase-Stimulating Protein,Dimerization Cofactor of HNF1,Dimerization Cofactor of Hepatocyte Nuclear Factor 1-α,Dimerization Cofactor of Hepatocyte Nuclear Factor 1-Alpha,DCoH,4-α-Hydroxy-Tetrahydropterin Dehydratase,4-Alpha-Hydroxy-Tetrahydropterin Dehydratase |
| 氨基酸序列 | Ala2-Thr104 |
| 蛋白构建 | Ala2-Thr104 |
| 分子量 | 14 KDa (reducing condition) |
| 内毒素 | < 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
| 缓冲液 | Supplied as a 0.2 μm filtered solution of 20 mM Tris-HCl, 150 mM NaCl, 1 mM DTT, pH 8.0. |
| 存储 | Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at -80°C. For reconstituted protein solutions, the solution can be stored at -20°C to -80°C for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots. |
| 运输方式 | In general, Lyophilized powders are shipping with blue ice. Solutions are shipping with dry ice. |
| 研究背景 | 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. |