6-pyruvoyltetrahydropterin synthase, ELISA Kit

Human 6-pyruvoyl tetrahydrobiopterin synthase (PTS) ELISA kit; FLJ97081; PTPS; 6-pyruvoyl tetrahydrobiopterin synthase; PTP synthase; 6-pyruvoyltetrahydropterin synthase

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of PTS elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9324984 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the 6-pyruvoyltetrahydropterin synthase (PTS) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing PTS. The ELISA analytical biochemical technique of the MBS9324984 kit is based on PTS antibody-PTS antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PTS antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PTS. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

16,386 Da

6-pyruvoyltetrahydropterin synthase

6-pyruvoyl tetrahydrobiopterin synthase; PTP synthase

6-pyruvoyl tetrahydrobiopterin synthase

PTP synthase; PTPS  [Similar Products]

PTPS_HUMAN

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. [provided by RefSeq, Oct 2008]

PTS: Involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. 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. Belongs to the PTPS family.

Protein type: EC 4.2.3.12; Mitochondrial; Cofactor and Vitamin Metabolism - folate biosynthesis; Lyase

Chromosomal Location of Human Ortholog: 11q22.3

Cellular Component: mitochondrion; cytoplasm; cytosol

Molecular Function: identical protein binding; protein homodimerization activity; metal ion binding; 6-pyruvoyltetrahydropterin synthase activity

Biological Process: amino acid metabolic process; tetrahydrobiopterin biosynthetic process; central nervous system development; regulation of nitric-oxide synthase activity; nitric oxide metabolic process

Disease: Hyperphenylalaninemia, Bh4-deficient, A

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