Dogs and Neurodegenerative Diseases

Neurodegenerative disease in dogs?? This question posed in discussion on Monday led me to ponder if such evidence had ever been found. On the website "Science Daily" I was able to find a review article published in August of this year titled, "Gene Responsible for Neurodegenerative Disease in Dogs, Possibly Humans, Discovered". The article was about Dr. Natashy Olby an associate professor of neurology at North Carolina State University who, along with a team of researchers, located a the gene responsible for a variant of Neuronal Ceroid Lipofuscinoses (NCL). My curiosity in exactly what NCL lead me to Web MD which describes NCL as a "group of neurodegenerative disorders" that are associated with variable yet progressive symptoms including seizures, dementia, visual loss, and/or cerebral atrophy. The pathophysiology of NCL is described as apoptosis and dysregulated sphingolipid metabolism. Although NCL, is rare in humans it is known as a disease in canines that results in mental and motor deterioration and eventually death in that affects American Staffordshire terriers. It is most common in children, although there is an adult-onset form called Kuf's disease. In this rare adult case, neurons in the brain die which then leads to loss of vision, epilepsy, dementia and loss of coordination. Researchers were able to locate this specific gene in canines through the use of genetic analysis. Being a review article, it did not go into detail as of how this genetic analysis was conducted. However, Obly claims that this mutation in canines is the novel nature of the mutation which means that researchers can now take and test samples from humans with NCL to determine whether this same mutation causes Kuf's disease in people. For more information on the pathophysiology of NCL and the mechanisms you can visit the Web MD site.

http://emedicine.medscape.com/article/1178391