Turnover of the fast components of myelin and myelin proteins in experimental hyperphenylalaninaemia. Relevance to termination of dietary treatment in human phenylketonuria

Abstract

The turnover of myelin and of myelin protein fractions has been measured in the central nervous system of rats who were placed on a hyperphenylalaninaemia-inducing diet (3% L-phenylalanine and 0.12% p-chlorophenylalanine added to the normal laboratory chow) when they were 25 days of age. A considerably increased turnover of the fast component of myelin and of myelin protein fractions was observed, which was not found in weight-matched controls or in controls fed the normal laboratory chow supplemented with 0.12% p-chlorophenylalanine. The increased turnover is therefore due to the hyperphenylalaninaemic condition and not due to the slow-down in growth or the presence of p-chlorophenylalanine. Furthermore, an inhibition of myelin synthesis due to the hyperphenylalaninaemic condition has been observed. Since these effects on myelin metabolism can be demonstrated to occur even when the brain has matured considerably, prudence should be exercised in considering the termination of the dietary treatment of patients with phenylketonuria.