[HTML][HTML] Stabilization of hyperdynamic microtubules is neuroprotective in amyotrophic lateral sclerosis

P Fanara, J Banerjee, RV Hueck, MR Harper… - Journal of Biological …, 2007 - ASBMB
Mutations in copper/zinc superoxide dismutase 1 (SOD1), a genetic cause of human
amyotrophic lateral sclerosis, trigger motoneuron death through unknown toxic mechanisms.
We report that transgenic SOD1G93A mice exhibit striking and progressive changes in
neuronal microtubule dynamics from an early age, associated with impaired axonal
transport. Pharmacologic administration of a microtubule-modulating agent alone or in
combination with a neuroprotective drug to symptomatic SOD1G93A mice reduced …