Human Dihydropteridine reductase ELISA Kit | QDPR elisa kit

Human Dihydropteridine reductase ELISA Kit

Cat. Num. AAA765719

• Gene Names: QDPR; DHPR; PKU2; SDR33C1
• Reactivity: Human
• Synonyms: Dihydropteridine reductase; Human Dihydropteridine reductase ELISA Kit; QDPR/DHPR/HDHPR/PKU2/SDR33C1/DHPR member 1/Dihydropteridine reductase/Quinoid Dihydropteridine Reductase; QDPR elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of QDPR. No significant cross-reactivity or interference between QDPR and analogues was observed.
• Sequence Length: 244

• Samples: Serum, plasma, tissue homogenates and other biological fluids
• Assay Type: Quantitative Sandwich
• Detection Range: 0.313-20ng/ml
• Sensitivity: 0.188ng/ml
• Intra-assay Precision: CV<8%
• Inter-assay Precision: CV<10%
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data/Standard Curve (for reference only)

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Related Product Information for QDPR elisa kit

Principle of the Assay: This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Capture antibody was pre-coated onto 96-well plates. And the biotin conjugated antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and washed with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the target amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of target can be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 12249123
• NCBI GeneID: 5860
• Molecular Weight: 22,408 Da
• NCBI Official Full Name: dihydropteridine reductase
• NCBI Official Synonym Full Names: quinoid dihydropteridine reductase
• NCBI Official Symbol: QDPR
• NCBI Official Synonym Symbols: DHPR; PKU2; SDR33C1
• NCBI Protein Information: dihydropteridine reductase
• UniProt Protein Name: Dihydropteridine reductase
• Protein Family: Dihydropteridine reductase
• UniProt Gene Name: QDPR
• UniProt Synonym Gene Names: DHPR; SDR33C1
• UniProt Entry Name: DHPR_HUMAN

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; Cofactor and Vitamin Metabolism - folate biosynthesis; Oxidoreductase

Chromosomal Location of Human Ortholog: 4p15.31

Cellular Component: cytoplasm; cytosol; mitochondrion; neuron projection

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

Biological Process: amino acid metabolic process; dihydrobiopterin metabolic process; L-phenylalanine catabolic process; liver development; response to aluminum ion; response to glucagon stimulus; response to lead ion; tetrahydrobiopterin biosynthetic process

Disease: Hyperphenylalaninemia, Bh4-deficient, C