Sensory Ataxic Neuropathy

Other Names: SAN
Affected Genes: tRNA-Tyr
Inheritance: Mitochondrial
Mutation: Deletion
Breed(s): Golden Retriever, Goldendoodle*
*Disease found in parent breed(s)

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Common Symptoms

Sensory ataxic neuropathy is an inherited neurologic condition affecting Golden Retrievers. Affected dogs typically present between 2 to 8 months of age with signs of neurologic disease. Symptoms include a lack of muscle coordination, abnormal gait and difficulty balancing especially affecting the hind limbs. Muscle mass appears normal and the condition does not appear to be painful. Although the disease progresses slowly, dogs are often humanely euthanized before three years of age.


Testing Tips

Genetic testing of the tRNA-Tyr gene will reliably determine whether a dog is a genetic Carrier of sensory ataxic neuropathy. Sensory ataxic neuropathy is a mitochondrial disorder. Dogs only inherit mitochondrial DNA from the mother. Because this disease is inherited in a maternal inheritance pattern, both male and female dogs must inherit the mutated gene from the mother to develop the disease. Affected male dogs do not pass their mitochondrial DNA to their offspring and cannot produce affected pups. Each pup that is born to a female dog carrying a Mutation in the tRNA-Tyr gene is at risk of having the disease. However, the ratio of mitochondria without the mutation to the number of mitochondria with the mutation is important in determining whether a specific dog will actually develop features of the disease. Reliable genetic testing is important for determining breeding practices. Because disease presentation is variable, genetic testing should be performed before breeding. In order to eliminate this mutation from breeding lines and to avoid the potential of producing affected pups, breeding of carrier or affected female dogs is not recommended. Dogs that are not carriers of the mutation have no increased risk of having affected pups.


References

  • Baranowska I, Jäderlund KH, Nennesmo I, Holmqvist E, Heidrich N, Larsson NG, Andersson G, Wagner EG, Hedhammar A, Wibom R, Andersson L. Sensory ataxic neuropathy in golden retriever dogs is caused by a deletion in the mitochondrial tRNATyr gene. PLoS Genet. 2009 May; 5(5):e1000499. [PubMed: 19492087]
  • Jäderlund KH, Orvind E, Johnsson E, Matiasek K, Hahn CN, Malm S, Hedhammar A. A neurologic syndrome in Golden Retrievers presenting as a sensory ataxic neuropathy. J Vet Intern Med. 2007 Nov-Dec; 21(6):1307-15. [PubMed: 18196741]