Studies in neuronal ceroid-lipofuscinosis: heterogeneous nature of neuronal autofluorescent lipopigments.

A method has been developed for the complete extraction and fractionation of the autofluorescent lipopigments present in the neuronal tissue of Batten-Spielmeyer-Vogt (BSV) patient and normal subject, respectively. The fluorescent lipopigments that were about three-fold higher in BSV patient's brain as compared with normal subjects were separated into at least 7 fluorometrically and chromatographically distinct fractions by thin layer chromatography (TLC). Although the fluorescent lipopigments of the brain tissue of BSV patient and normal subject showed identical pattern of separation on TLC, the flourescence spectra of the individual lipopigment fractions from BSV patient was different from those of the corresponding fractions from the normal subject. The ultraviolet spectral studies with individual fluorescent lipopigment fractions indicated the presence of highly unsaturated conjugated chromophore.