Recombinant Human PHS/PCBD1/Pterin-4-α-Carbinolamine Dehydratase
规格:
10ug/50ug/500ug/1mg
规格:
10ug
产品价格:
¥1200.0
规格:
50ug
产品价格:
¥3520.0
规格:
500ug
产品价格:
询价
规格:
1mg
产品价格:
询价
Recombinant Human PHS/PCBD1/Pterin-4-α-Carbinolamine Dehydratase (C246)
产品说明(Description)
Recombinant Human Pterin-4-alpha-Carbinolamine Dehydratase is produced by our E.coli expression system and the target gene encoding Ala2-Thr104 is expressed with a 6His tag at the N-terminus. Accession #: P61457 Known as: Pterin-4-Alpha-Carbinolamine Dehydratase; PHS; 4-Alpha-Hydroxy-Tetrahydropterin Dehydratase; Dimerization Cofactor of Hepatocyte Nuclear Factor 1-Alpha; DCoH; Dimerization Cofactor of HNF1; Phenylalanine Hydroxylase-Stimulating Protein; Pterin Carbinolamine Dehydratase; PCD; PCBD1; DCOH; PCBD
制剂(Formulation) Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, 1mM DTT, pH 8.0.
质量控制(Quality Control)
Purity: Greater than 95% as determined by reducing SDS-PAGE. Endotoxin: Less than 0.1 ng/ug (1 EU/ug) as determined by LAL test.
运输(Shipping) The product is shipped on dry ice/polar packs. Upon receipt, store it immediately at the temperature listed below.
保存(Storage) Store at ≤-70°C, stable for 6 months after receipt. Store at ≤-70°C, stable for 3 months under sterile conditions after opening. Please minimize freeze-thaw cycles.
背景(Background) 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.