Lysosomal Storage Disease

Description

Lysosomal storage diseases occur due to inherited deficiencies of various lysosomal enzymes, either due to abnormalities in its synthesis, processing or cellular trafficking. The normal function of lysosomes is to degrade molecules within the cell and, when these chemical reactions are truncated by the absence of a key enzyme, the substrates of the reaction accumulate within the organelle. Since there is some redundancy in the pathways by which proteins are degraded, deficiencies in proteases do not usually result in clinical disease.

There are a large number of different lysosomal storage diseases in dogs and cats categorised according to the product which accumulates within cells. All of the storage disease are rare.