Abstract— The uptake into brain and the incorporation into brain protein of intraperitoncally administered, labelled amino acids has been studied in myelinating rats during prolonged hyperphenylalaninaemia maintained by administration of p-chlorophenylalanine. Compared with controls, there was a 50% reduction in both uptake and incorporation into protein of leucine and a parallel reduction in the acid-soluble leucine pool. With glycine and lysine no such changes were observed. On the other hand, when each of the three amino acids was injected directly into the brain, the only significant differences observed between controls and hyperphenylalaninaemic animals were again with leucine, which showed an increased incorporation into protein and an increased specific activity in the otherwise reduced acid-soluble pool.
It is concluded that hyperphenylalaninaemia reduces the rate of transport of leucine into the brain and hence reduces the brain pool of leucine, but that any effects on protein synthesis are small. The validity of the model, and the implications of the findings, in relation to phenylketonuria, are discussed.