PCBD1 (pterin-4 alpha-carbinolamine dehydratase), also known as PCD, PHS, DCOH (dimerization cofactor of hepatocyte nuclear factor 1 alpha) or PCBD, is a component of the phenylalanine hydroxylase (PAH) system and participates intetrahydrobiopterin biosynthesis.
More specifically, PCBD1 catalyzes the dehydration of pterin-4alpha-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-1alpha.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.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of PCBD1. Species Crossreactivity: Human, mouse, rat
