| Catalog# |
C246 |
| Source |
E.coli |
| Description |
Recombinant Human Pterin-4-α-Carbinolamine Dehydratase/PCBD1 is produced by our E. coli expression system. The target protein is expressed with sequence (Ala2-Thr104) of Human PCBD1 fused with a His tag at the N-terminus. |
| Names |
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 Carbinolami |
| Accession # |
P61457 |
| Formulation |
Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, 1mM DTT, pH 8.0 |
| Shipping |
The product is shipped on dry ice/ice packs. |
| Storage |
Store at < -20°C, stable for 6 months after receipt.
Please minimize freeze-thaw cycles. |
| Purity |
Greater than 95% as determined by reducing SDS-PAGE. |
| Endotoxin |
Less than 0.1 ng/μg (1 IEU/μg). |
| Amino Acid Sequence |
MGSSHHHHHHSSGLVPRGSHMAGKAHRLSAEERDQLLPNLRAVGWNELEGRDAIFKQFHFKDFNR AFGFMTRVALQAEKLDHHPEWFNVYNKVHITLSTHECAGLSERDINLASFIEQVAVSMT
|
| 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. |
