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Product Name
Anti-PTS antibody
- Documents
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Description
Rabbit polyclonal to PTS
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Tested applications
ELISA, IHC-P
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Species reactivity
Human PTS
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Alternative names
6-pyruvoyltetrahydropterin synthase antibody; PTS antibody; PTPS antibody; 6-pyruvoyl tetrahydrobiopterin synthase antibody; PTP synthase antibody; 6-pyruvoyl-tetrahydropterin synthase antibody; Pts antibody; PTPS antibody; 6-pyruvoyl tetrahydrobiopterin synthase antibody; PTP synthase antibody
- Immunogen
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Isotype
Rabbit IgG
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Preparation
Produced in rabbits immunized with purified, recombinant Human PTS (rh PTS; Q03393; Met1-Glu145). PTS specific IgG was purified by Human PTS affinity chromatography.
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Clonality
Polyclonal
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Formulation
0.2 μm filtered solution in PBS
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Storage instructions
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free.
Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles. -
Applications
ELISA:0.1-0.2 μg/mL
This antibody can be used at 0.1-0.2 μg/mL with the appropriate secondary reagents to detect Human PTS. The detection limit for Human PTS is < 0.039 ng/well.
IHC-P: 0.1-1 μg/ml
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Validations
PTS Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
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Background
PTS(6-pyruvoyltetrahydropterin synthase) belongs to the PTPS family. It 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. PTS is involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. PTS also catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6-pyruvoyl tetrahydropterin. Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A), also called 6-pyruvoyl-tetrahydropterin synthase deficiency (PTS deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to PTS deficiency. HPABH4A is an autosomal recessive disorder characterized by depletion of the neurotransmitters dopamine and serotonin, and clinically by severe neurological symptoms unresponsive to the classic phenylalanine-low diet.
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References
- Ashida A, et al. (1994) A missense mutation (A to G) of 6-pyruvoyltetrahydropterin synthase in tetrahydrobiopterin-deficient form of hyperphenylalaninemia. Genomics. 24:408-10.
- Ashida A, et al. (1993) cDNA cloning, expression in Escherichia coli and purification of human 6-pyruvoyl-tetrahydropterin synthase. Biochem. Biophys Res Commun. 195:1386-93.
- Thoeny B, et al. (1992) Human 6-pyruvoyltetrahydropterin synthase: cDNA cloning and heterologous expression of the recombinant enzyme. Biochem Biophys Res Commun. 189:1437-43.
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