{"product_id":"new-product-281708","title":"PCBD1 polyclonal antibody","description":"\u003cp\u003ePCBD1 (pterin-4 α-carbinolamine dehydratase), also known as PCD, PHS, DCOH (dimerization cofactor of hepatocyte nuclear factor 1 α) or PCBD, is a component of the phenylalanine hydroxylase (PAH) system and participates in\u003cbr\u003e\ntetrahydrobiopterin biosynthesis. More specifically, PCBD1 catalyzes the dehydration of pterin-4α-carbinolamine (4-OH-BH4) to quinonoid dihydrobiopterin(q-BH2), an essential reaction for the regeneration of 6(R)-L-erythro-5,6,7,8-tetrahydrobiopterin (6(R)BH4). In addition, PCBD1 can homodimerize and, in this dimer, can function as a transcriptional activator cofactor for HNF-1α.Mutations in the gene encoding PCBD1 lead to an accumulation of 4-OH-BH4 which subsequently produces 7-BH4 (a potent inhibitor of PAH), and may result in primapterinuria. Patients with primapterinuria (a mild form of phenylketonuria (PKU) exhibit both hyperphenylalaninemia (HPA) and excretion of 7-substituted pterins.\u003c\/p\u003e","brand":"ABCbiolab","offers":[{"title":"100ul \/ Rabbit \/ Human,Mouse,Rat","offer_id":45809051205912,"sku":"ABCBW09798","price":408.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/758-7580448_monoclonal-antibody-png-transparent-png_6c6f8b98-d214-4d0a-bf3c-e17bfa2f8f15.png?v=1689755035","url":"https:\/\/danabiosci.com\/products\/new-product-281708","provider":"Dana Bioscience","version":"1.0","type":"link"}