pterin-4-a-carbinolamine dehydratase

"的结果

重组蛋白
1

对比

PHS Protein, Human, Recombinant (His)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

TMPJ-01043

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.