17 March 2011. Amyotrophic lateral sclerosis is a disease not
only of lower motor neurons but also of the upper motor neurons
(UMNs), but whether UMN degeneration in mouse models closely
mirrors human pathology has been unclear. In today's Journal of
Neuroscience, researchers outline a detailed characterization of
the neural degeneration that occurs in the brain of one type of
ALS mouse model. Hande Ozdinler of Northwestern University in
Evanston, Illinois, who led the study in collaboration with her
former advisor Jeffrey Macklis of the Massachusetts General
Hospital in Boston, reports that accompanying lower motor neuron
deficits, there is early and specific loss of upper motor
neurons in these animals. The findings may give researchers a
handle on degeneration of those same neurons in humans.
Superoxide dismutase-1 (SOD1), when mutated, can cause ALS in
people, and mice carrying human SOD1-G93A are a common model for
the disease. Scientists knew that the mice, in addition to their
well-known spinal cord mo