In 2002, three related Golden Retrievers with gait abnormalities were presented to the author. The clinical picture of those dogs was not in agreement with any other previously described canine disease. Since then, more than 20 Golden Retriever dogs have been diagnosed with this novel neurological syndrome by the author. Both the phenotype and the genotype have been published (1,2) The disease was characterized as a mitochondrially inherited sensory ataxic neuropathy owing to the neurological signs,in agreement with a similar clinical syndrome in humans. Sensory ataxic neuropathies present with loss of proprioceptive sensations and tendon reflexes, with preservation of muscle strength (3). This syndrome is associated with a number of aetiologies inhumans and mitochondrial disorders are recognized among them. The canine disease is suitable to serve as a naturally occurring animal model for mitochondrial diseases.
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