Alaskan Husky encephalopathy / AHE (SLC19A3-related) - Alaskan Husky

What does this test examine?

This genetic test examines the SLC19A3 c.624 insTTGC variant known in Alaskan Husky encephalopathy, abbreviated AHE. The condition is also described within the spectrum of subacute necrotising encephalopathy or Leigh-like encephalopathy.

What does this mean for the dog?

SLC19A3 encodes a thiamine transporter that is important for the brain and nervous system. Dogs with two copies of the variant can develop severe neurological signs, including ataxia, balance problems, seizures, altered mentation, swallowing difficulty, impaired vision and paralysis. The disorder can start at a young age and can be severe.

Practical value of this test

• The test distinguishes clear dogs, carriers and genetically affected dogs.
• Breeders can manage carriers deliberately and avoid pairings that may produce affected puppies.
• For Alaskan Husky lines, the result gives concrete information about a serious inherited neurological disorder.

The condition is inherited as an autosomal recessive trait. Carriers usually do not have AHE, but can pass the variant on; two copies cause the disorder.