Rat Dihydropteridine reductase (QDPR) ELISA Kit | QDPR elisa kit
Rat Dihydropteridine reductase (QDPR) ELISA Kit
Principle of the Assay: QDPR ELISA kit applies the competitive enzyme immunoassay technique utilizing an anti-QDPR antibody and an QDPR-HRP conjugate. The assay sample and buffer are incubated together with QDPR-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the QDPR concentration since QDPR from samples and QDPR-HRP conjugate compete for the anti-QDPR antibody binding site. Since the number of sites is limited, as more sites are occupied by QDPR from the sample, fewer sites are left to bind QDPR-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The QDPR concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the enzyme dihydropteridine reductase, which catalyzes the NADH-mediated reduction of quinonoid dihydrobiopterin. This enzyme is an essential component of the pterin-dependent aromatic amino acid hydroxylating systems. Mutations in this gene resulting in QDPR deficiency include aberrant splicing, amino acid substitutions, insertions, or premature terminations. Dihydropteridine reductase deficiency presents as atypical phenylketonuria due to insufficient production of biopterin, a cofactor for phenylalanine hydroxylase. [provided by RefSeq, Jul 2008]
Uniprot Description
QDPR: The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases. Defects in QDPR are the cause of BH4-deficient hyperphenylalaninemia type C (HPABH4C); also called dihydropteridine reductase deficiency (DHPR deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to DHPR deficiency or quinoid dihydropteridine reductase deficiency (QDPR deficiency). HPABH4C is a rare autosomal recessive disorder characterized by hyperphenylalaninemia and severe neurologic symptoms (malignant hyperphenylalaninemia) including axial hypotonia and truncal hypertonia, abnormal thermogenesis, and microcephaly. These signs are attributable to depletion of the neurotransmitters dopamine and serotonin, whose syntheses are controlled by tryptophan and tyrosine hydroxylases that use BH-4 as cofactor. These patients do not respond to phenylalanine- restricted diet. HPABH4C is lethal if untreated. Belongs to the short-chain dehydrogenases/reductases (SDR) family.
Protein type: EC 1.5.1.34; Oxidoreductase; Cofactor and Vitamin Metabolism - folate biosynthesis
Chromosomal Location of Human Ortholog: 4p15.31
Cellular Component: neuron projection; mitochondrion; cytoplasm; cytosol
Molecular Function: protein homodimerization activity; electron carrier activity; 6,7-dihydropteridine reductase activity
Biological Process: amino acid metabolic process; L-phenylalanine catabolic process; tetrahydrobiopterin biosynthetic process; response to glucagon stimulus; dihydrobiopterin metabolic process; response to lead ion; liver development; response to aluminum ion
Disease: Hyperphenylalaninemia, Bh4-deficient, C
Research Articles on QDPR
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Product Notes
The Rat QDPR qdpr (Catalog #AAA7242119) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7242119 ELISA Kit recognizes Rat QDPR. It is sometimes possible for the material contained within the vial of "Dihydropteridine reductase (QDPR), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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