Pterin-4-?-Carbinolamine Dehydratase (PCBD1) is the founding member of the Pterin-4-?-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.
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.
Purity:
> 95 % as determined by reducing SDS-PAGE.
Mol Mass:
14.2 kDa
AP Mol Mass:
14 kDa
Formulation:
Supplied as a 0.2 µm filtered solution of 20mM TrisHCl, 150mM NaCl, 1mM DTT, pH 8.0.
Shipping:
This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs.Upon receipt, store it immediately at<-20°C.
Stability and Storage:
Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.
