Canine Model of ALS Emerges


by Kathlyn Stone

January 12, 2009

Researchers have found that dogs with a neurodegenerative disorder share a gene mutation similar to that for amyotrophic lateral sclerosis (ALS) in humans. The finding could one day boost the development of potential ALS therapies.

Certain dog breeds are prone to degenerative myelopathy, which eventually can cause paraplegia. Historically, these dogs, often larger breeds, were euthanized because their size made care difficult.

But then the disease began showing up in smaller breeds such as boxers and Welsh corgis. Pet owners nursed their dogs longer and the disease further progressed, allowing researchers to learn that these dogs have a mutation in the SOD1 gene, similar to the ALS mutation in humans.

“When we found the mutation in SOD1, we found lower motor neuron involvement and it was really sounding like ALS,” said Dennis O'Brien, a veterinary neurologist at the University of Missouri College of Veterinary Medicine.

O’Brien and Joan Coates, a colleague at Missouri who has studied dogs and degenerative myelopathy for a decade, are now working with breeders and the American Kennel Club to test dogs for the mutation.

Studies are under way at Missouri and collaborating veterinary medicine centers to see if there are decreased motor neuron numbers in the ventral horns of dogs in the advanced stages of degenerative myelopathy.

Future therapies for ALS may be tested on these dogs, similar to chemotherapy trials conducted in canines with cancer ahead of human clinical research.

The study is accepted for publication in the Proceedings of the National Academy of Sciences.