Article
Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALS.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 23 Aug 2006
Gould Thomas W, Buss Robert R, Vinsant Sharon, Prevette David, Sun Woong, Knudson C Michael, Milligan Carol E, Oppenheim Ronald W
Abstract excerpt
The death of cranial and spinal motoneurons (MNs) is believed to be an essential component of the pathogenesis of amyotrophic lateral sclerosis (ALS). We tested this hypothesis by crossing Bax-deficient mice with mice expressing mutant superoxide dismutase 1 (SOD1), a transgenic model of familial ALS. Although Bax deletion failed to prevent neuromuscular denervation and mitochondrial vacuolization, MNs were...
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