ID #1301

Can you briefly explain lysosomal storage disorders?

A lysosomal storage disorder is caused by a deficiency in one of the acid hydrolases in a lysosome. Glucocerebrosidase is an enzyme found in a lysosome. A person who has Gaucher's disease (one of the 45 known lysosomal storage disorders) is lacking functional glucocerebrosidase. This causes accumulations of non-degraded products, and interferes with normal cell metabolism, and eventually in tissue function. The most commonly affected tissues are nervous tissue, so these storage disorders usually present as nervous disorders.

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