BS8181

antibody from Bioworld Technology, Inc
Targeting: PCBD1 DCOH, PCBD, PCD
Provider product page for BS8181
Western blot
Immunohistochemistry

Antibody data

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.
PCBD1 protein structure - BS8181 shown in red.