Dihydropteridine reductase; HDHPR; Quinoid dihydropteridine reductase; Qdpr; Dhpr; 1.5.1.34

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

For long term storage, please store the entire kit at -20 degree C.

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Small volumes of Qdpr 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.

MBS289110 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Dihydropteridine reductase (Qdpr) 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 Qdpr. The ELISA analytical biochemical technique of the MBS289110 kit is based on Qdpr antibody-Qdpr antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect Qdpr antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, Qdpr. 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(%).

25,570 Da

quinoid dihydropteridine reductase

dihydropteridine reductase

Dihydropteridine reductase

Dhpr??[Similar Products]

DHPR_MOUSE

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

Cellular Component: cytoplasm; cytosol; mitochondrion; neuron projection

Molecular Function: 6,7-dihydropteridine reductase activity; oxidoreductase activity; protein homodimerization activity

Biological Process: metabolic process; tetrahydrobiopterin biosynthetic process

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