Degenerative Myelopathy and Lou Gehrig’s Disease

January 22, 2009 at 6:20 am 6 comments

Today Genetic Enginerring & Biotechnology News reported that researchers from University of Missouri and the Broad Institute have discovered that the genetic mutation responsible for degenerative myelopathy (DM) in dogs is the same one that causes Lou Gehrig’s disease (amyotrophic lateral sclerosis or ALS) in humans.

“We uncovered the genetic mutation of degenerative myelopathy, which has been unknown for 30 years, and linked it to ALS, a human disease that has no cure,” said Joan Coates, a veterinary neurologist and associate professor of veterinary medicine and surgery in the MU College of Veterinary Medicine. “Dogs with DM are likely to provide scientists with a more reliable animal model for ALS. Also, this discovery will pave the way for DNA tests that will aid dog breeders in avoiding DM in the future.”

That DNA test would be a blessing. Six years ago we had to let our Aussie Roo go when DM completely robbed her of the strength and agility she was known for in her youth. Words can’t describe how difficult it is to watch your dog get weaker and less coordinated every day — and worst of all, to be utterly helpless to stop it.

         Aussie Roo
Aussie Roo

DM and ALS are progressive neurodegenerative diseases that affect nerve cells in the brain and the spinal cord.  As the motor neurons atrophy and harden, muscle weakness occurs, especially in the arms and legs.  As the disease progresses and motor neurons die, all ability to initiate and control muscle movements is lost.

There is no cure for either disease and DM typically progresses so rapidly that most dogs are euthanized within a year of diagnosis.

Comparative medicine is the study of the similarities and differences in disease patterns in different mammals. Dogs are particularly useful to study because they share our environment, and are similar to us in body size.  Parallels in the diseases that affect humans and our dogs can provide benefits to both species.  Studies conducted on dogs can help develop treatments and genetic screening tests for humans — and these drugs and tests can also be used to improve the health of dogs.

Previously, ALS research has relied heavily on transgenic rodents that expressed the mutant human gene SOD1, which causes ALS. Researchers found that dogs with DM also had mutations in their SOD1 gene. Many rodent models possess very high levels of the SOD1 protein that can produce pathologic processes distinct from those occurring in ALS patients. Since the SOD1 mutation is spontaneous in dogs, the clinical spectrum in dogs may represent more accurately that of human ALS.

“Compared with the rodent models for ALS, dogs with DM are more similar to people in size, structure and complexity of their nervous systems, and duration of the disease,” said Gary Johnson, associate professor of veterinary pathobiology in the MU College of Veterinary Medicine. “The results from clinical trials conducted with DM-affected dogs may better predict the efficacies of therapeutic interventions for treating ALS in humans.”

The staff at the Broad Institute are doing some wonderful work in conjunction with the canine genome project.  Please visit their website and, if your dog is eligible, consider participating in their research.  They need DNA samples from both healthy and diseased dogs and they can’t do this work without our help. 

We gave them blood samples and other information when Zorro died last spring – the old man’s many health problems made him particularly interesting to them.  They were kind and helpful, sympathetic to our loss and generous in the help they provided our vet.  Knowing that the information they got from his DNA might help cure or prevent the diseases he suffered from was one small comfort we could take in that difficult time.

The cost of a blood draw is small is tiny compared to the great good it may do.  Be generous.  That blood might help save the life of a dog – or person – you love someday.

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Entry filed under: dogs, health, science. Tags: , .

Dog vs Technology – Round II Breakfast of Champions

6 Comments Add your own

  • 1. Joe  |  January 22, 2009 at 1:12 pm

    I have to echo the importance of this research, both for our dogs and on the human front. When we were going through the DM diagnostics with my Chessie, Emma, I took her to Dr. Coates very early in the process. When I became suspicious of DM I wanted her seen by the best. She is an amazing individual, and I hope her research is a gateway into a better understanding of how these terrible diseases work so that a cure or treatment can someday be found.

    I think it is so easy for us to think someone else will find the answer, or provide the samples, for these researchers to do their job. At the end of the day they absolutely need support from dog owners in order to have success. As owners we need to be aware of conditions unique to our breeds and be willing to offer any assistance we can through donations, sample collection, health survey information, etc. This research, while benefiting to our canine friends, can also lead to lifesaving research for us as well.

    The one silver-lining I take away from Emma’s condition was that she played a role in this research and her genetic material aided in developing a test so that others may not have to watch their best friend suffer from this horrendous condition.

    On the human side of the equation I could not think of a crueler disease to have than to end up trapped in your body with a completely sharp mind. Maybe someday this key research will lead to a cure, or successful treatments, for those afflicted by this disease.

    When we knew that Emma only had a limited amount of time I vowed to do everything I could to allow her to continue to do what she loved, even if her body could no longer perform at the level she had known. Here are a couple of pix from our last hunting season together with her hunting from a wheelchair:

  • 2. Audie's Gramma  |  January 22, 2009 at 5:38 pm

    ALS, huh?

    I’d always wondered if it wasn’t the same as MS, but did not seriously look into it.

    Now we know.

  • 3. Dorene  |  January 22, 2009 at 8:44 pm

    Oh, well. So much for those of us with mutts. Did send the URL out on my dog park list as there are plenty of folks there with purebreds

  • 4. SmartDogs  |  January 22, 2009 at 10:54 pm

    Yes but — please realize that these people aren’t being purebred snobs. The reduced genetic variation between dogs of the same breed is a huge advantage to researchers as it narrows the number of places to search for the alleles controlling genetic diseases.

    And hey – your mutt *will* benefit from these studies if screening tests or treatments are found.

  • 5. Dorene  |  January 23, 2009 at 2:34 am

    Yeah, I understood that. I was just bummed that Pepper couldn’t contribute more. She’s actually pretty good about letting the vet take a blood sample — unlike getting her nails clipped (go figure!), so i was all ready to sign her up.

  • 6. Blondie'sMom  |  August 9, 2012 at 12:09 pm

    My white GSD was diagnosed with the MS form of DM. Primary Progressive MS is a very rare, little known form of MS for which there is no treatment. My girl has lost the abilty to feel me touching her and that is not associated with ALS. She is also very heat sensitive now as a result of the disease and that is also not associated with ALS. The signs and symptoms of PPMS and ALS are nearly identical, but the diseases are different. tremor, swallowing difficulty, breathing issues, sleep disturbances, speech issues and the like can occur in PPMS and ALS. The jury is still out on this. There have been “clear” dogs who have had confirmed DM as well as carriers who have had confrimed DM. The SOD1 gene is not the smoking gun that it intially was thought to be. It’s too early in the study to be sure of anything but what I do know is that there is more than one form of DM, my dog was diagnosed with the MS form and sadly the U of M does not recognize this oversight.

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