|Other Names||Sepiapterin reductase, SPR, SPR|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Catalyzes the final one or two reductions in tetra- hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.|
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Provided below are standard protocols that you may find useful for product applications.
SPR 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.
Leu-Semenescu, S., et al. Sleep 33(3):307-314(2010)Schnetz-Boutaud, N.C., et al. Genes Brain Behav. 8(8):753-757(2009)Clot, F., et al. Brain 132 (PT 7), 1753-1763 (2009)
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