Sapropterin dihydrochloride (6R-BH4 dihydrochloride) (Synonyms: BH4) |
| Catalog No.GC32438 |
Sapropterin ((6R)-BH4) dihydrochloride is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations.
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Cas No.: 69056-38-8
Sample solution is provided at 25 µL, 10mM.
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Sapropterin dihydrochloride is a synthetic form of BH4 that is approved for the treatment of BH4 responsive PKU. (1) Sapropterin dihydrochloride can stimulate TH and TPH activities leading to improved dopamine and serotonin synthesis despite persistently elevated brain phenylalanine.(2) Sapropterin dihydrochloride is used to lower blood phenylalanine levels in tetrahydrobiopterin-responsive phenylketonuria in conjunction with a phenylalanine-restricted diet.
[1]. Winn SR et al. High dose sapropterin dihydrochloride therapy improves monoamine neurotransmitter turnover in murine phenylketonuria (PKU). Mol Genet Metab. 2016 Jan;117(1):5-11. [2]. Somaraju UR et al. Sapropterin dihydrochloride for phenylketonuria. Cochrane Database Syst Rev. 2015 Mar 27 [3]. Jurecki ER et al. Sapropterin Dihydrochloride Mixed With Common Foods and Beverages. Top Clin Nutr. 2014 Oct;29(4):325-331. [4]. Grange DK et al. Sapropterin dihydrochloride use in pregnant women with phenylketonuria: an interim report of the PKU MOMS sub-registry. Mol Genet Metab. 2014 May;112(1):9-16
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