Antibody data
- Antibody Data
- Antigen structure
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- Validations
- Western blot [1]
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- Product number
- BS8181 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- PCBD1 polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- Recombinant full length Human PCBD1.
- Description
- PCBD1 (pterin-4 Ï«-carbinolamine dehydratase), also known as PCD, PHS, DCOH (dimerization cofactor of hepatocyte nuclear factor 1 Ï«) or PCBD, is a component of the phenylalanine hydroxylase (PAH) system and participates in tetrahydrobiopterin biosynthesis. More specifically, PCBD1 catalyzes the dehydration of pterin-4Ï«-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-1Ï«.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.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
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Supportive validation
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- Bioworld Technology, Inc (provider)
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- Experimental details
- WesternBlot (WB) analysis of PCBD1 polyclonal antibody