PCBD1 polyclonal Antibody | BS8181

PCBD1 polyclonal Antibody | BS8181 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse,Rat

Application: WB IF

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

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.

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

Specificity: PCBD1 polyclonal Antibody detects endogenous levels of PCBD1 protein.

Molecular Weight: ~ 12 kDa

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

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

Immunogen: Recombinant full length Human PCBD1.

Conjugate: Unconjugated

Modification: Unmodification

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) .

Pathway: