PHS Protein, Human, Recombinant (His)

Ref. TM-TMPJ-01043

Produktinformation

• Name: PHS Protein, Human, Recombinant (His)
• Synonyme: 4-Alpha-Hydroxy-Tetrahydropterin Dehydratase, Pterin-4-α-Carbinolamine Dehydratase, Phenylalanine Hydroxylase-Stimulating Protein, Dimerization Cofactor of Hepatocyte Nuclear Factor 1-Alpha, PHS, Dimerization Cofactor of HNF1, Dimerization Cofactor of Hepatocyte Nuclear Factor 1-α, 4-α-Hydroxy-Tetrahydropterin Dehydratase, DCoH, Pterin Carbinolami, Pterin-4-Alpha-Carbinolamine Dehydratase

Beschreibung

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.

• Marke: Targetmol

Chemische Eigenschaften

• Molekulargewicht: 14 KDa (reducing condition)
• Farbe/Form: Solid