Storage Diseases in Dogs
What is a storage disease?
These diseases are more accurately known as lysosomal storage diseases, and refer to a group of rare inherited metabolic disorders that result from defects in the function of lysosomes. Lysosomes are the "recycling center" in cells, and are supposed to process unwanted or worn out material in a cell into a substance that the cell can use. The lysosomes use enzymes to do this processing, and if the enzyme exists in only small amounts or is absent altogether, the recycling process doesn't work, and the unprocessed material builds up, or is stored, in the cell. Eventually the stored material builds up so much that the cell cannot function any longer. When this happens in enough cells, disease symptoms appear.
What symptoms are typical of a storage disease?
The symptoms of lysosomal storage diseases vary, depending on what type of cell is malfuntioning and what material is being stored. Some have a relatively early age of onset, others may not appear until middle or old age, and symptoms range from fairly mild, to severe and deadly. Storage disease symptoms can include developmental delay, movement disorders, seizures, dementia, deafness, and blindness.
Several types of Neuronal Ceroid Lipofuscinosis have been the focus of research here at the University of Missouri for many years, and this website has a section devoted this type of lysosomal storage disease. CLICK HERE to go to the NCL website.
Recent work has identified the mutation responsible for GM2-Gangliosidosis in Japanese Chin, and a new DNA test is now available - CLICK HERE for information on gangliosidosis. When research is launched on other storage diseases in dogs, we will add more information to this website.
This website is designed to provide basic information on canine storage diseases, and serve as a resource for those concerned with these diseases: breeders, owners, veterinarians, and researchers.
Information presented here is not a substitute for an accurate diagnosis and specific advice geared to your pet's specific needs. If you suspect your dog has a storage disease or is showing symptoms suggestive of a storage disease, we encourage you to have a veterinarian or veterinary neurologist examine the dog and advise you. If you need to locate a veterinary neurologist CLICK HERE to search for a specialist near you.
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