Recombinant Human PCBD1 Protein (His Tag)

SKU: PKSH032981

Size: 50μg

Storage: Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.

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.

Exp date: 12 months

Category ID_II: Recombinant Proteins

Category ID_III: Others

Abbreviation: PCBD1

Target Synonym: 4-Alpha-Hydroxy-Tetrahydropterin Dehydratase;DCoH;Dimerization Cofactor of HNF1;Dimerization Cofactor of Hepatocyte Nuclear Factor 1-Alpha;PHS;Phenylalanine Hydroxylase-Stimulating Protein;Pterin Carbinolamine Dehy;Pterin-4-Alpha-Carbinolamine Dehydratase

Research Areas: Signal Transduction;epigenetics and nuclear signal;metabolism;

Conjugation:

Target Species: Human

Expression Host: E.coli

Application:

Fusion tag: N-His

UNIProt ID: P61457

Accession: P61457

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.

Concentration:

Activity: Not validated for activity

Sequence: Ala2-Thr104

Purity: > 95 % as determined by reducing SDS-PAGE.

Formulation: Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, 1mM DTT, pH 8.0.

Reconstitution: Not Applicable

Endotoxin: < 1.0 EU per μg of the protein as determined by the LAL method.

Calculated MW: 14.2 kDa

ObservedMW: 14 kDa