I. Introductory concepts

 D. Examples of Lysosomal Storage Diseases with Neurological Involvement

•  Neuronal storage diseases, e.g. Tay-Sachs disease and Niemann-Pick disease type A ---characterized primarily by storage of uncleaved substrate in neurons and other cell types
• Leukodystrophies, e.g. Krabbe disease and metachromatic leukodystrophy ---characterized by impairment of a biochemical process necessary for myelin development or maintenance

Schematic representation of the compartments of an animal cell, showing organelles including a lysosome, which is the site of degradation of many types of molecules.   From McGill Molson Medical Informatics Project
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