PCBD1 polyclonal, anti-human, mouse, rat
€388.00
In stock
SKU
BS8181
Background:
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 in tetrahydrobiopterin 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.
Alternative Name:
Pterin-4-alpha-carbinolamine dehydratase, PHS, 4-alpha-hydroxy-tetrahydropterin dehydratase, Dimerization cofactor of hepatocyte nuclear factor 1-alpha, DCoH, Dimerization cofactor of HNF1, Phenylalanine hydroxylase-stimulating protein, Pterin carbinolamine dehydratase, PCD, PCBD1, DCOH, PCBD
Application Dilution: WB: 1:500~1:2000, IF: 1:50~1:200
Specificity: PCBD1 polyclonal antibody detects endogenous levels of PCBD1 protein.
Immunogen:
Recombinant full length Human PCBD1.
MW: ~ 12 kDa
Swis Prot.: P61457
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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 in tetrahydrobiopterin 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.
Alternative Name:
Pterin-4-alpha-carbinolamine dehydratase, PHS, 4-alpha-hydroxy-tetrahydropterin dehydratase, Dimerization cofactor of hepatocyte nuclear factor 1-alpha, DCoH, Dimerization cofactor of HNF1, Phenylalanine hydroxylase-stimulating protein, Pterin carbinolamine dehydratase, PCD, PCBD1, DCOH, PCBD
Application Dilution: WB: 1:500~1:2000, IF: 1:50~1:200
Specificity: PCBD1 polyclonal antibody detects endogenous levels of PCBD1 protein.
Immunogen:
Recombinant full length Human PCBD1.
MW: ~ 12 kDa
Swis Prot.: P61457
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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