- 中文名稱
PCBD1 polyclonal antibody
- 英文名字
- PCBD1 polyclonal antibody
- 供應商
- Bioworld
- 產品貨號
- BS8181
- 產品報價
- ¥詢價/50ul

- 產品說明書
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- 購買方式
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- 產品新聞

- 背景資料
- PCBD1 (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 inntetrahydrobiopterin 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.
- 應用類型
- WB, IF/ICC
- 免疫原
- Recombinant full length Human PCBD1.
- 來源宿主
- Rabbit
- 反應性
- Human, Mouse, Rat
- 保存建議
- Store at 4
- 其他
- Bioworld是專一服務于生命科學研究的專業技術型企業,Bioworld品牌創立以來擁有10000多種常規產品現貨,現有8000多種現貨抗體,以及其他IVD原料,蛋白等現貨產品。在細胞信號通路、免疫學、蛋白組學上擁有顯著優勢,其抗體產品、生長因子、IVD原料等為生命科學科研工作者提供了極大的便利

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