Abstract
High levels of phenylalanine inhibit the central nervous system specific ATP-sulfurylase, leading to a decreased synthesis of sulfatides. The hypothesis is developed that this in turn leads to an increased turnover of central nervous system myelin which is not compensated by an increased rate of synthesis. As a consequence, synaptic contacts regress, resulting in fewer stable synaptic contacts, which is a contribution to brain dysfunction observed in not treated or poorly treated PKU patients.
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© 1988 Birkhäuser Boston
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Hommes, F.A., Matsuo, K. (1988). Effect of Phenylalanine on Brain Maturation: Implications for the Treatment of Patients with Phenylketonuria. In: Wurtman, R.J., Ritter-Walker, E. (eds) Dietary Phenylalanine and Brain Function. Birkhäuser Boston. https://doi.org/10.1007/978-1-4615-9821-3_28
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DOI: https://doi.org/10.1007/978-1-4615-9821-3_28
Publisher Name: Birkhäuser Boston
Print ISBN: 978-1-4615-9823-7
Online ISBN: 978-1-4615-9821-3
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