Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Sepiapterin reductase (SPR) Standard Curve.)
Human Sepiapterin Reductase (SPR) ELISA Kit | SPR elisa kit
Human Sepiapterin Reductase (SPR) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Sepiapterin reductase (SPR) Standard Curve.)
Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Sepiapterin reductase (SPR) Standard Curve.)
Background: Sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase; EC 1.1.1.153) catalyzes the NADPH-dependent reduction of various carbonyl substances, including derivatives of pteridines, and belongs to a group of enzymes called aldo-keto reductases. SPR plays an important role in the biosynthesis of tetrahydrobiopterin (BH4). The clone encoded a protein of 261 amino acids with a calculated molecular mass of 28,047 Da. The predicted amino acid sequence of human sepiapterin reductase shows 74% identity with the rat enzyme and a striking homology with human carbonyl reductase, estradiol 17-beta-dehydrogenase, and 3-beta-hydroxy-5-ene steroid dehydrogenase, especially in the N-terminal region.
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: Cofactor and Vitamin Metabolism - folate biosynthesis; EC 1.1.1.153; Oxidoreductase
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: L-phenylalanine metabolic process; tetrahydrobiopterin biosynthetic process; voluntary musculoskeletal movement; death; pteridine metabolic process; neuron morphogenesis during differentiation; regulation of multicellular organism growth; regulation of nitric-oxide synthase activity; nitric oxide biosynthetic process; norepinephrine metabolic process; dopamine metabolic process; nitric oxide metabolic 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 #AAA9714010) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9714010 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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