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The enzyme encoded by PTS catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Additionally we are shipping PTS Antibodies (60) and PTS Proteins (14) and many more products for this protein.
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Disruption of PTPS Gene Causing Pale Body Color and Lethal Phenotype in the Silkworm, Bombyx mori.
Severe neurological impairment from BH4 deficiency could be prevented by newborn screening for hyperphenylalaninemia (HPA) and proper metabolic management.
A total of 43 mutations were identified in the PTS gene in a screen of East Asian populations, comprising 22 previously reported mutations and 21 new discovered mutations.
The mutant characterization of PTPS gene was coincident with other early studies in Chinese. The novel mutation L127F was considered as a pathogenetic mutation and associated with severe clinical phenotype.
Our data show that PTPS induction is necessary for optimized BH4 synthesis in cytokine-stimulated human coronary artery endothelial cells and point to IL-1beta as a leading cytokine in this process.
Hyperphenylalaninemia may be caused by deficiency of Phe hydroxylase or by deficiency of co-factor BH(4).
Human PTS was efficiently expressed in noradrenergic regions but only in a small number of dopaminergic neurons.
Expression of PTS was significantly decreased in PD cases.
We suggest that the serum prolactin level may be a more sensitive marker than the CSF HVA level to guide the dose adjustment of L-Dopa/carbidopa in the management of patients with PTPS deficiency.
mutational analysis in Chinese hyperphenylalaninemia caused by tetrahydrobiopterin synthesis deficiency
The enzyme encoded by this gene catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Tetrahydrobiopterin, also known as BH(4), is an essential cofactor and regulator of various enzyme activities, including enzymes involved in serotonin biosynthesis and NO synthase activity. Mutations in this gene result in hyperphenylalaninemia.
, 6-pyruvoyl tetrahydrobiopterin synthase
, putative 6-pyruvoyltetrahydropterin synthase
, PTP synthase