UC Davis School of Veterinary Medicine Veterinary Genetics Laboratory
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Alaskan Husky Encephalopathy
Introduction

Alaskan Husky Encephalopathy (AHE) is a brain disease affecting Alaskan Husky (AH) dogs. The disease usually affects juvenile and young adult males and females, and often multiple dogs from the same litter. The disease is eventually fatal, although some dogs may live for months to years before the signs progress. Signs of AHE include seizures, trouble walking, a high stepping gait, trouble eating, and visual problems. AHE is inherited as a simple autosomal recessive trait. The disease potentially can occur in any breed, but is most commonly found in the Alaskan Husky. A DNA test for the specific AHE mutation is now available that can determine if dogs are normal, or if they carry one or two copies of the defective gene. Dogs that carry two copies of the mutation are affected and expected to develop neurological problems.

The VGL offers a DNA test for AHE to assist owners and breeders in identifying affected and carrier dogs. The test uses DNA collected from buccal (cheek) swabs, thus avoiding blood sample collection. Breeders can use results from the test as a tool for selection of mating pairs to avoid producing affected dogs.

Female

Male

N/N

N/AHE

AHE/AHE

N/N

100% N/N

50% N/N, 50% N/AHE

100% N/AHE

N/AHE

50% N/N, 50% N/AHE

25% N/N, 50% N/AHE, 25% AHE/AHE

50% N/AHE, 50% AHE/AHE

AHE/AHE

100% N/AHE

50% N/AHE, 50% AHE/AHE

100% AHE/AHE

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Allow 2-6 business days for results.

Results reported as:

N/N: no copies of AHE mutation; dog is normal
N/AHE: 1 copy of AHE mutation; dog is normal but is a carrier
AHE/AHE: 2 copies of AHE mutation; dog is affected. Age of onset of neurological problems is variable.

Research on AHE is ongoing in Dr. Danika Bannasch’s laboratory at UC Davis to identify other breeds with this problem. If the dog has the mutation then treatment modalities may be used to treat the dog.

References

Brenner, O., et al., Alaskan Husky encephalopathy--a canine neurodegenerative disorder resembling subacute necrotizing encephalomyelopathy (Leigh syndrome). Acta Neuropathol (Berl), 2000. 100(1): p. 50-62.

Wakshlag, J.J., et al., Subacute necrotising encephalopathy in an Alaskan husky. J Small Anim Pract, 1999. 40(12): p. 585-9.

Vernau KM, JA Runstadler, EA Brown et al. 2013. Genome-Wide Association Analysis Identifies a Mutation in the Thiamine Transporter 2 (SLC19A3) Gene Associated with Alaskan Husky Encephalopathy. Plos One 8(3):e57195.  

 
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