The development of the central nervous system is highly dependent on an adequate supply of nutrients. In particular, protein and amino acid availability is of major concern during gestation and in early postnatal life. Numerous data have been published on some amino acids directly involved in brain functions as neurotransmitters or indirectly as precursors of neurotransmitters, but scant information is available on the possible consequences of hyperthreoninemia, a phenomenon repeatedly noted in clinical reports. The results of neurochemical and behavioral studies in the developing rat suggest that despite numerous possible effects of threonine on brain constituents, moderate hyperthreoninemia does not impair markedly the development of the central nervous system.