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Journal of pediatric endocrinology & metabolism : JPEM • 2017 | View Paper
“The rate of phosphorylation of human phenylalanine hydroxylase was inhibited about 40% by the cofactor tetrahydrobiopterin , and this inhibition was completely prevented by the simultaneous presence of L-phenylalanine (i.e. at turnover conditions).”
Archives of biochemistry and biophysics • 1996 | View Paper
“Thus, phosphorylation of the enzyme by cAMP-dependent protein kinase is stimulated by phenylalanine , especially in the presence of BH4 , (which by itself inhibits), whereas phosphorylation sensitizes the enzyme to activation by phenylalanine.”
Journal of pediatric endocrinology & metabolism : JPEM • 2016 | View Paper
“… 6R-tetrahydrobiopterin … phenylalanine intake (from 49.1 [25.6-60.3] to 56.5 [39.8-68.3] mgkg(-1)day(-1)) and natural protein intake (….8-1.7] to 1.5 [1.0-1.8] g kg(-1)day(-1)), and some patients managed to adopt normal diets.”
Molecular genetics and metabolism • 2015 | View Paper
“BACKGROUND Sapropterin dihydrochloride BH4 , tetrahydrobiopterin) can lower plasma phenylalanine (Phe) concentrations for a subset of patients with phenylketonuria (PKU), an inborn error of metabolism.”
Molecular genetics and metabolism • 2013 | View Paper
“On long-term BH4 treatment over a period of 48 ± 27 months in a dose of 14.9 ± 3.3mg/kg/day phenylalanine tolerance was increased from 452 ± 201 mg/day to 1593 ± 647 mg/day, corresponding to a mean increase of 1141 ± 528 mg/day.”
Molecular genetics and metabolism • 2012 | View Paper
“Treatment with tetrahydrobiopterin (BH4), the natural cofactor of phenylalanine hydroxylase (PAH), can reduce blood phenylalanine (Phe) levels in patients with BH4-responsive phenylketonuria (PKU).”