Effects of hyperphenylalaninemia in the fetal stage on the postnatal development of fetal rat brain.

The effect of exposure at different prenatal stages to maternal hyperphenylalaninemia (HyPhe) on the somatic and neurological development of fetuses in rats was studied, with special respect to the change of relevant enzyme activities in the brain. While evident somatic damage was found only in the fetuses exposed to maternal HyPhe at a last stage of gestation, distinct mental retardation seemingly due to some irreversible damage to the brain was observed in all the treated fetuses regardless of the timing of exposure, and a significantly reduced activity of 2', 3'-cyclic nucleotide 3'-phosphohydrolase (CNPase), a marker enzyme of myelin, was confirmed in the mantle region of the brain.