?PKU Bodies?: Characteristic inclusions in the brain in phenylketonuria

Abstract

The ultrastructure of various inclusions within oligodendroglial cells in the brains from two phenylketonuric patients was studied. Characteristic lamellar, oval, slightly irregular inclusions measured between 0.5 and 2 micra in diameter and were bounded by a single membrane. The longitudinal and transverse lamellae of these inclusions had a distinct pattern. In analogy to lamellated but different inclusions of other diseases, these structures were termed “PKU bodies”. Various possibilities that would explain the morphogenesis of the PKU bodies and other inclusions in the oligodendroglial cells, and the significance of these findings, are discussed.