PHS Protein, Human, Recombinant (His)

Product Overview

• Article Name: PHS Protein, Human, Recombinant (His)
• Biozol Catalog Number: TGM-TMPJ-01043
• Supplier Catalog Number: TMPJ-01043
• Alternative Catalog Number: TGM-TMPJ-01043-1MG,TGM-TMPJ-01043-5UG,TGM-TMPJ-01043-10UG,TGM-TMPJ-01043-20UG,TGM-TMPJ-01043-50UG,TGM-TMPJ-01043-100UG,TGM-TMPJ-01043-200UG,TGM-TMPJ-01043-500UG
• Manufacturer: TargetMol
• Category: Biochemikalien
• Alternative Names: 4-Alpha-Hydroxy-Tetrahydropterin Dehydratase , Pterin-4-alpha-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-alpha , 4-alpha-Hydroxy-Tetrahydropterin Dehydratase , DCoH , Pterin Carbinolami , Pterin-4-Alpha-Carbinolamine Dehydratase

Product Description

Pterin-4-alpha-Carbinolamine Dehydratase (PCBD1) is the founding member of the Pterin-4-alpha-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.

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TMPJ-01043