IMMUNOLOGICAL STUDIES ON SPHINGOLIPID ACTIVATOR PROTEINS IN THE NEURONAL, CEROID-LIPOFUSCINOSES

The neuronal ceroid-lipofuscinoses constitute an important group of pr ogressive encephalopathies leading to severe psychomotor retardation, blindness, and early death. They are characterized by accumulation of autofluorescent, electron-dense storage bodies within the cytoplasm of neurons and many other cell types. WE! have recently identified sphin golipid activator proteins A and D as major components of the storage cytosomes in the infantile form of NCL. Using an immunological approac h we have shown that sphingolipid activator proteins also constitute a n integral component of the storage bodies in the other major forms of the disease.