Glycoconjugates in storage cytosomes from ceroid-lipofuscinosis (Batten's disease) and in lipofuscin from old-age brain

Advances in Experimental Medicine and Biology
N A HallA D Patrick

Abstract

The ceroid-lipofuscinoses (CL) are a group of inherited diseases characterised by the accumulation, in brain, of autofluorescent storage cytosomes which have similar histochemical staining properties to lipofuscin, the neuronal wear and tear pigment of old-age brain. The storage cytosomes stain strongly with periodic acid-Schiff reagent (PAS), indicating the presence of carbohydrate. In brain from each childhood form of CL, concentrations of phosphorylated dolichol (Dol-P) are 10- to 20- fold higher than in age-matched controls. Brain Dol-P concentrations are also increased between 2 and 5- fold in several different lipidoses and in elderly subjects. Much of the Dol-P which accumulates is located within the storage cytosomes. Dol-P constitutes 2-3% of the dry weight of storage cytosomes from juvenile and late-infantile CL, and 0.3-0.7% of storage cytosomes from infantile CL, ovine CL and of lipofuscin isolated from old age brain. The bulk of the Dol-P in CL brain and in isolated storage cytosomes is present as dolichyl pyrophosphoryl oligosaccharides (Dol-PP-OS). The constitutions of the oligosaccharide moieties differ in the various forms of the disease. Histochemical analysis of frozen sections of unfixed brain after extracti...Continue Reading

Citations

Jun 1, 1994·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·W A DunnE A Brown
Jan 1, 1993·Journal of Inherited Metabolic Disease·R K Pullarkat, S E Zawitosky

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Batten Disease

Batten Disease is a group of nervous system disorders known as neuronal ceroid lipofuscinosis. This feed focuses on neurobiological and neuropathological aspects of this disease.