[Lysosomal proteolysis; overview of lysosomal diseases]

The lysosome plays a key role in recycling other cell organelles and long-lived cellular proteins, which undergo continuous turnover. Lying at the hub of vesicular pathways, the functions of which are tightly inter-regulated, the proteolytic machinery of the lysosome is essential for cellular homeostasis. Lysosomal diseases are rare genetic disorders due to deficiencies in one or several lysosomal protein(s). Their clinical manifestations are extremely diverse and depend on the total or partial nature of the protein deficiency and the specific functions of this organelle in different cell types.