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.

Specificity:

PCBD1 polyclonal antibody detects endogenous levels of PCBD1 protein.

Alternative names:

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

Species reactivity:

Human,Mouse,Rat

Host:

Rabbit

Ig type:

Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Immunogen:

Recombinant full length Human PCBD1.

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).

Molecular weight:

~ 12 kDa

Applications:

WB IF

Usage:

WB: 1:500~1:2000 IF: 1:50~1:200

Storage:

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Precautions:

For research use only, not for use in diagnostic procedure.

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