ELEVATED LEVELS OF DOLICHOL IN THE BRAINS OF MUCOPOLYSACCHARIDOSIS AND RELATED DISORDERS

The contents of total dolichol were measured in the cerebral cortex of various patients with lysosomal storage disorders, including mucopoly saccharidosis. Strikingly high levels of dolichol were demonstrated in G(M1)-gangliosides, Sanfilippo B syndrome, and a severe type of Hunte r syndrome as well as neuronal ceroid-lipofuscinosis. An increased lev el of dolichol in cerebral cortex in neuronal ceroid-lipofuscinosis (N CL) was once regarded as pathognomonic for NCL. Our data, however, sug gest that an increased level of dolichol in cerebral cortex is a nonsp ecific phenomenon related to some lysosomal dysfunction secondary to v arious neurodegenerative disorders.