Article
Motor unit abnormalities in Dystonia musculorum mice.
PloS one - 1 Jan 2011
De Repentigny Yves, Ferrier Andrew, Ryan Scott D, Sato Tadasu, Kothary Rashmi
Abstract excerpt
Dystonia musculorum (dt) is a mouse inherited sensory neuropathy caused by mutations in the dystonin gene. While the primary pathology lies in the sensory neurons of dt mice, the overt movement disorder suggests motor neurons may also be affected. Here, we report on the contribution of motor neurons to the pathology in dt(27J) mice. Phenotypic dt(27J) mice display reduced alpha motor neuron cell number and...
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