GM1 Gangliosidosis

Signs include partial or total vision loss, behavior changes, abnormal gait, and seizures.

This is an early onset form of lysosomal storage disease, this can cause affected dogs to display neurologic signs as puppies or young adults.

Genetic and laboratory testing used to diagnose this disease. Advanced imaging (MRI/CT) may be used to exclude other similar disease processes.

There is no treatment except supportive care for the clinical signs the affected dog develops.

Talk to your vet about your dog’s GM1 gangliosidosis result so you can work together to plan ongoing care and monitoring. Note any changes in their coordination, strength, or behavior.

Keep your dog’s environment calm and consistent. Use slings, harnesses, or supportive devices as needed to help with mobility, and place rugs or mats on slippery floors to prevent falls.

Encourage gentle, food-based activities such as treat searches or puzzle toys to help your dog stay mentally engaged without overexertion.

Help your dog feel secure by keeping furniture in familiar places, using non-slip mats, and maintaining predictable daily routines.

Types of gangliosidosis are defined by the enzyme deficiency that causes the disease. GM1 gangliosidosis is caused by mutations in the GLB1 gene, which codes for the enzyme beta-galactosidase. GLB1 mutations have been identified in affected Shiba Inus, Alaskan Huskies, and Portuguese Water Dogs.

Penetrance: This disease is inherited in an autosomal recessive manner, meaning that affected dogs must have two copies of the mutation to show clinical signs.

Gene

GLB1 (Exon 15)

Inheritance type

recessive

Clinical category

Multisystem

Citations

1. Kreutzer et al 2005

• Alaskan-type Husky
• Siberian Husky