Typical Testing Data/Standard Curve (for reference only)
Human Sepiapterin Reductase (SPR) ELISA Kit | SPR elisa kit
Human Sepiapterin Reductase (SPR) ELISA Kit
To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
Principle of the Assay: The microplate provided in this kit has been pre-coated with an antibody specific to SPR. Standards or samples are then added to the appropriate microplate wells with a biotin-conjugated antibody specific to SPR. Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain SPR, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm +/- 10nm. The concentration of SPR in the samples is then determined by comparing the O.D. of the samples to the standard curve.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an aldo-keto reductase that catalyzes the NADPH-dependent reduction of pteridine derivatives and is important in the biosynthesis of tetrahydrobiopterin (BH4). Mutations in this gene result in DOPA-responsive dystonia due to sepiaterin reductase deficiency. A pseudogene has been identified on chromosome 1. [provided by RefSeq, Jul 2008]
Uniprot Description
SPR: Catalyzes the final one or two reductions in tetra- hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin. Defects in SPR are the cause of dystonia DOPA-responsive due to sepiapterin reductase deficiency (DRDSPRD). In the majority of cases, patients manifest progressive psychomotor retardation, dystonia and spasticity. Cognitive anomalies are also often present. The disease is due to severe dopamine and serotonin deficiencies in the central nervous system caused by a defect in BH4 synthesis. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Belongs to the sepiapterin reductase family.
Protein type: Oxidoreductase; Cofactor and Vitamin Metabolism - folate biosynthesis; EC 1.1.1.153
Chromosomal Location of Human Ortholog: 2p14-p12
Cellular Component: nucleoplasm; mitochondrion; cytoplasm; cytosol
Molecular Function: aldo-keto reductase activity; sepiapterin reductase activity; NADP binding
Biological Process: voluntary musculoskeletal movement; tetrahydrobiopterin biosynthetic process; death; pteridine metabolic process; regulation of multicellular organism growth; norepinephrine metabolic process; nitric oxide metabolic process; dopamine metabolic process; L-phenylalanine metabolic process; neuron morphogenesis during differentiation; regulation of nitric-oxide synthase activity; nitric oxide biosynthetic process; serotonin metabolic process
Disease: Dystonia, Dopa-responsive, Due To Sepiapterin Reductase Deficiency
Research Articles on SPR
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Product Notes
The Human SPR spr (Catalog #AAA2020241) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2020241 ELISA Kit recognizes Human SPR. It is sometimes possible for the material contained within the vial of "Sepiapterin Reductase (SPR), 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
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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