Recombinant Human Pterin-4-α-Carbinolamine Dehydratase/PHS/PCBD1

Instruction Manual!

Product name:	Recombinant Human Pterin-4-α-Carbinolamine Dehydratase/PHS/PCBD1
Source:	E.coli
Purity:	Greater than 95% as determined by reducing SDS-PAGE.
Buffer Formulation:	Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, 1mM DTT, pH 8.0.
Applications:	Applications:SDS-PAGE; WB; ELISA; IP.
Storage:	Avoid repeated freeze/thaw cycles. Store at 2-8 oC for one month. Aliquot and store at -80 oC for 12 months.
UOM:	100ug/50ug/200ug/1mg/1g

Source	E.coli
Description	Recombinant Human PCBD1 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.
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 Carbinolamine Dehydratase, PCD, PCBD1, DCOH, PCBD
Accession #	P61457
Formulation	Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 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) as determined by LAL test.
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.