Cure to ALS found in dogs?

Internal medicine board-certified neurologist Joan Coates, DVM, MS and a team of researchers from the University of Missouri (MU) and the Broad Institute have found the genetic mutation responsible for degenerative myelopathy (DM) in dogs is the same mutation that causes amyotrophic lateral sclerosis (ALS), the human disease also known as Lou Gehrig’s Disease.

“We uncovered the genetic mutation of DM which has been unknown for 30 years,” said Dr. Coates. “Dogs with DM are likely to provide scientists with another animal model for ALS. Since DM is a spontaneous disease in dogs, it may represent a more reliable model than genetically modified mice for biomarkers and possible therapeutic strategies.”

DM, a progressive disease of the spinal cord, causes affected dogs to wobble when walking and has a high prevalence in German Shepherds, but is also found in Cardigan and Pembroke Welsh Corgis, Rhodesian Ridgebacks, Chesapeake Bay Retrievers, and Boxers. Although it is not a painful disease, weakness gets progressively worse until the dog is unable to walk and becomes paraplegic. ALS is a progressive neurodegenerative disease in humans that attacks nerve cells in the brain and spinal cord resulting in muscle weakness and atrophy.

Dr. Coates, an associate professor of veterinary medicine and surgery at MU, began studying DM in dogs in 1997 when on faculty at Texas A&M University. Over the course of a decade, Dr. Coates and a team of researchers, including Drs. Gary Johnson, Claire Wade, Kerstin Lindblad-Toh, and their colleagues, embarked on a research path which required the collection of many cases and samples from many dog breeders, owners, and veterinarians. e ultimate result was the discovery that DM is caused by a gene mutation in the superoxide dismutase 1 (SOD1) gene, which is the same gene that causes some forms of ALS in humans. Even with this extraordinary discovery, the road to curing both diseases is far from over. “The outlook for a dog with DM is still grave,” said Dr. Coates. According to Dr. Coates, efforts are being made to improve quality of life such as good nursing care, physical rehabilitation, pressure sore prevention, monitoring of urinary infections, and ways to increase mobility through the use of harnesses and carts for affected dogs.

“We still have a lot of work to do in finding ways to follow disease progression so that we can monitor therapeutic strategies,” said Dr. Coates. “Hopefully this discovery will offer an opportunity to other veterinary neurologists and specialists to use their expertise for further study of this disease and in establishing ‘proof of concept’ for possible therapeutic strategies.”