Glucose-lowering effect of beta-phenylpyruvate in neonatal mice: a possible mechanism for phenylketonuria-related neurodegenerative changes.

Following beta-phenylpyruvate injection, mice developed hypoglycemia clinically manifested as tachypnea, tremor, convulsions and death. To further investigate, neonatal mice were injected with beta-phenylpyruvate and their blood glucose determined and brain histology assessed. beta-Phenylpyruvate-injected mice exhibited higher mortality and neurophysiological changes as compared with controls, although without evidence of neural cell death. Accordingly, we hypothesize that the central neural damage in phenylketonuria might be caused by these recurrent beta-phenylpyruvate-induced hypoglycemic events. Copyright 2003 Elsevier Science B.V.