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Studies in neuronal ceroid-lipofuscinosis: Heterogeneous nature of neuronal autofluorescent lipopigments

✍ Scribed by Hari S. Garg; Yogesh C. Awasthi; Satish K. Srivastava

Publisher
John Wiley and Sons
Year
1981
Tongue
English
Weight
800 KB
Volume
6
Category
Article
ISSN
0360-4012

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✦ Synopsis

Abstract

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 fluorescence 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.

πŸ“œ SIMILAR VOLUMES

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✍ Rider, J. Alfred ;Dawson, Glyn ;Siakotos, Aristotle N. πŸ“‚ Article πŸ“… 1992 πŸ› John Wiley and Sons 🌐 English βš– 659 KB

## Abstract The search for biochemical abnormalities in the neuronal ceroid‐lipofuscinoses (NCL) or Batten disease was initiated with the discovery of normal levels of gangliosides in juvenile amaurotic idiocy. The primary goal of most biochemical studies has been to discover the unique biochemical