Article
Overview of immune abnormalities in lysosomal storage disorders.
Immunology letters - 1 Aug 2017
Rigante Donato, Cipolla Clelia, Basile Umberto, Gulli Francesca, Savastano Maria Cristina
Abstract excerpt
The critical relevance of the lysosomal compartment for normal cellular function can be proved by numbering the clinical phenotypes that arise in lysosomal storage disorders (LSDs), a group of around 70 different monogenic autosomal or X-linked syndromes, caused by specific lysosomal enzyme deficiencies: all LSDs are characterized by progressive accumulation of heterogeneous biologic materials in the lysosomes of...
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