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• W1977548787 startingPage "1143" @default.
• W1977548787 abstract "Summary: Purpose: Phenylketonuria (PKU) is a disorder of phenylalanine (Phe) metabolism that frequently results in epilepsy if a low Phe diet was not implemented at birth. The mechanisms by which Phe affects the brain are poorly understood. Methods: Audiogenic seizures (AGS) were studied in female homozygous Pah enu2 BTBR (PKU) mice. Results: Adult PKU mice, 18–20 weeks of age, in contrast to wild‐type and heterozygous counterparts, exhibited a full range of AGS. Younger PKU mice, 5–7 weeks of age, had higher serum Phe levels (2.22 ± 0.20 mM) in comparison with the adult animals (1.72 ± 0.05 mM) and were not susceptible to AGS. Among adult mice, animals susceptible to AGS had significantly lower serum Phe levels (1.62 ± 0.06 mM) in comparison with those resistant to AGS (1.86 ± 0.07 mM). Susceptibility to AGS tended to increase in the afternoon when serum Phe concentration decreased in comparison to evening and morning. Normalization of serum Phe level by instituting a low Phe diet generally prevented susceptibility to AGS within 12 h. Although return to a standard diet raised Phe levels to hyperphenylalaninemic within 12 h in animals treated with a low Phe diet for 2 weeks, more than 7 weeks were needed for a complete resumption of AGS. Conclusions: Transient decrease in Phe levels within hyperphenylalaninemic range may be a necessary condition for PKU‐related seizures to occur. A low Phe diet prevents susceptibility to seizures, which can resume with the significant delay after termination of dietary treatment." @default.
• W1977548787 created "2016-06-24" @default.
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• W1977548787 date "2007-05-01" @default.
• W1977548787 modified "2023-10-17" @default.
• W1977548787 title "Epilepsy in Phenylketonuria: A Complex Dependence on Serum Phenylalanine Levels" @default.
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• W1977548787 doi "https://doi.org/10.1111/j.1528-1167.2007.01115.x" @default.
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