Phenylalanine and phenylacetate adversely affect developing mammalian brain neurons

Kenneth F. Swaiman, Si Ru Wu

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

Abstract

Myelin alteration is thought to be the primary pathologic characteristic of phenylketonuria. Clinical symptoms and histologic changes in brain suggest that neuronal insult also may be important. We evaluated the effects of chronic exposure of phenylalanine (0.6 mM) and phenylacetate (0.6 mM) on immature mammalian cortical neuronal cultures. Observations after exposure suggested neuronal drop out. 125Itetanus toxin binding, choline acetyltransferase activity, high-affinity 3H-GABA uptake, and glutamic acid decarboxylase activity decreased in the presence of either metabolite. Neither substance was a more potent cause of adverse effects. Chronic exposure to either phenylalanine or phenylacetate had a detrimental effect on cultured cortical neurons, both cholinergic and GABAergic.

Original languageEnglish (US)
Pages (from-to)1246-1250
Number of pages5
JournalNeurology
Volume34
Issue number9
StatePublished - Sep 1984

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