The tetrahydrobiopterin (BH4) cofactor is essential for hepatic hydroxylase, which is involved in phenylalanine degradation and catecholamine and serotonin biosynthesis.
BH4 is also an essential and limiting cofactor for all types of nitric oxide synthase.
BH4 deficiency results in hyperphenylalaninemia andmonoamine neurotransmitter depletion and is most commonly due to autosomal recessive mutation in 6-pyruvoyltetrahydropterin synthase (PTPS), the second enzyme for BH4 biosynthesis.
The active site of PTPS consists of thepterin-anchoring Glu A107 neighbored by two catalytic motifs: a Zn(II) binding site and an inter-subunit catalytic triad formed by Cys A42, Asp B88 and His B89.
The active site of PTPS undergoes a Zn and Mg-dependent reaction thatincludes a triphosphate elimination, a stereospecific reduction and the oxidation of both side hydroxyl groups.
The catalytic triad of PTPS is involved in the deprotonation of the side-chain carbons of substrates.
In addition, Ser 19 of human PTPS may be a substrate for cGMP-dependent protein kinase type IIphosphorylation in vivo, which is essential for normal activity of PTPS.
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 PTS. Species Crossreactivity: Human, mouse, rat
