Paper Alert: A Canine Model of ALS

Dogs who suffer from degenerative myelopathy carry a mutation in the gene for superoxide dismutase 1 (SOD1), the same gene that is defective in 20 percent of familial amyotrophic lateral sclerosis (ALS) cases, according to a paper in this week’s PNAS online. ARF first covered this research, by Joan Coates, Dennis O’Brien, and colleagues at the University of Missouri in Columbia and elsewhere, when it was presented at the November 2008 Society for Neuroscience meeting in Washington, DC (see Nov 2008 news).

Degenerative myelopathy (DM) shares symptoms with ALS; both are late-onset, progressive, fatal neurodegenerative diseases. Like ALS patients with SOD1 mutations, the animals exhibit SOD1-positive inclusions in spinal cord sections. The authors suggest that DM could be a useful animal model for ALS, allowing scientists to follow progression and test therapies in a naturally occurring disease.

Tomoyuki A, Johnson GS, Wade CM, Katz ML, Johnson GC, Taylor JF, Perloski M, Biagi T, Baranowska I, Long S, March PA, Olby NJ, Shelton GD, Khan S, O’Brien DP, Lindblad-Toh K, Coates JR. Genome-wide association analysis reveals a SOD1 mutation in canine degenerative myelopathy: A model for amyotrophic lateral sclerosis. PNAS 2009 Feb 24;106(8):2794-9. [Pubmed].

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