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Molecular genetics and metabolism • 2021 | View Paper
“Sapropterin dihydrochloride sapropterin ) lowers blood Phe in tetrahydrobiopterin ( BH4 ) responsive patients with PKU, in conjunction with a Phe-restricted diet, but clinical evidence supporting its use during pregnancy is limited.”
Molecular genetics and metabolism • 2014 | View Paper
“Plasma concentrations of BH4 were significantly elevated 3 h after ingestion of sapropterin (0 h: 19.1 ± 2 pmol/ml vs. 3 h: 43.8 ± 3 pmol/ml; P < 0.001).”
“Treatment with sapropterin resulted in clinically significant and sustained reductions in blood Phe concentrations and increased dietary Phe tolerance in well-designed clinical studies in PKU patients who responded to BH4.”
Expert opinion on drug metabolism & toxicology • 2013 | View Paper
“Supplementation of BH4 either by intraperitoneal administration of sepiapterin or of 6RBH4 ( (6R)-l-erythro-5,6,7,8-tetrahydrobiopterin ) increased the BH4 content in the intestinal lumen with a slight delay after the rise of blood BH4.”