Lysosomal Storage Disease

Lysosomes are cell particles that function in intracellular digestive processes. They are found in most cells but are particularly prominent in leukocytes and the cells of liver and kidney. Lysosomes have several distinct appearances that can vary based on cell type and activation status including spheroid, ovoid, or tubular. In addition, the size of lysosomes can vary dramatically.

Mutated lysosomal enzymes are the underlying cause of a number of lysosomal storage diseases. Over 30 human inherited lysosomal storage diseases, with additional subtypes, have been described in which specific substances accumulate in lysosomes as a result of certain defects. Many of these diseases have also been reported in domestic animals.


Credit: PubMed Central


  1. Distinct Lysosome Phenotypes Influence Inflammatory Function in Peritoneal and Bone Marrow-Derived Macrophages
  2. Lysosomes in iron metabolism, ageing and apoptosis