RT Journal Article
SR Electronic
T1 Phenylalanine and phenylacetate adversely affect developing mammalian brain neurons
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 1246
OP 1246
DO 10.1212/WNL.34.9.1246
VO 34
IS 9
A1 Swaiman, Kenneth F.
A1 Wu, Si Ru
YR 1984
UL http://n.neurology.org/content/34/9/1246.abstract
AB 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. 125I- tetanus 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.