Gangliosidosis
Metabolic/Digestive • GLB1 • Recessive
Summary
Gangliosidosis is an inherited neurological disorder seen in Shiba Inus and caused by a mutation in the beta-galactosidase gene. This mutation prevents the normal breakdown of certain lipids that are vital for nerve cell function. As these lipids accumulate, nerve cells become damaged and begin to die, leading to severe neurological decline. The condition follows an autosomal recessive pattern, meaning puppies must inherit the defective gene from both parents to be affected.
Symptoms
Symptoms usually begin within the first six months of life. Affected dogs may develop progressive vision loss, unsteady balance, weight loss, and head tremors. As the disease advances, neurological function deteriorates rapidly, leaving dogs increasingly debilitated. Most affected Shiba Inus do not survive beyond 18 months of age.
Care
There is currently no cure or effective treatment for gangliosidosis. Veterinary care is supportive only, focusing on comfort and safety, but due to the progressive and debilitating nature of the disease, many dogs are euthanized to prevent ongoing suffering.
Citations
Wang, Z.H., Zeng, B., Shibuya, H., Johnson, G.S., Alroy, J., Pastores, G.M., Raghavan, S., Kolodny, E.H. : Isolation and characterization of the normal canine beta-galactosidase gene and its mutation in a dog model of GM1-gangliosidosis. J Inherit Metab Dis 23:593-606, 2000. Pubmed reference: 11032334. DOI: 10.1023/a:1005630013448.
Review
28/08/2025 by Dr Robyn Bird
Breed
Portuguese water dog