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Lysosomal storage diseases (LSDs) are mainly caused by the defective activity of lysosomal hydrolases. A sub-class of LSDs are the sphingolipidoses, in which sphingolipids accumulate intra-cellularly. We here discuss the role of innate immunity in the sphingolipidoses, and compare the pathways of activation in two classical sphingolipidoses, namely Gaucher disease and Sandhoff disease, and in Niemann-Pick C disease, in which the main storage material is cholesterol but sphingolipids also accumulate. We discuss the mechanisms leading to neuroinflammation, and the different pathways of neuroinflammation in the different diseases, and suggest that intervention in these pathways may be a useful therapeutic approach to address these devastating human diseases.

Original publication

DOI

10.1515/hsz-2014-0301

Type

Journal article

Journal

Biol Chem

Publication Date

06/2015

Volume

396

Pages

659 - 667

Keywords

Animals, Brain, Gaucher Disease, Humans, Immunity, Innate, Lysosomal Storage Diseases, Niemann-Pick Disease, Type C, Sphingolipidoses