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Therapeutic brain modulation with targeted large neutral amino acid supplements in the Pah-enu2 phenylketonuria mouse model
phenylketonuria inborn error of metabolism large neutral amino acids mouse model neurotransmitters brain biochemistry treatment
2018/12/18
Background: Phenylketonuria treatment consists mainly of a Phe-restricted diet, which leads to suboptimal neurocognitive and psychosocial outcomes. Supplementation of large neutral amino acids (LNAAs)...
Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial
inborn errors of amino acid metabolism phenylalanine tyrosine threonine medical food sapropterin dihydrochloride executive function
2018/12/3
Background: To prevent cognitive impairment, phenylketonuria requires lifelong management of blood phenylalanine (Phe) concentration with a low-Phe diet. The diet restricts intake of Phe from natural ...