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Updated: Aug 18, 2026

Expanding the Toolkit for In Vivo Imaging of Axonal Transport
Published on: December 23, 2021
Mutant superoxide dismutase disrupts cytoplasmic dynein in motor neurons
Lee A Ligon1, Bernadette H LaMonte, Karen E Wallace
1Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
Abstract:
Cytoplasmic dynein and dynactin drive retrograde axonal transport in neurons, and mutations in dynein/dynactin cause motor neuron degeneration. To test whether defects in dynein/dynactin function are involved in the neurodegenerative disease amyotrophic lateral sclerosis, we examined neurotracer transport from muscle to motor neuron in a transgenic mouse model of amyotrophic lateral sclerosis. Significant inhibition was observed, which was temporally correlated with declines in muscle strength. No decrease in dynein/dynactin expression was observed, but immunohistochemistry suggests that dynein associates with aggregates of mutant Cu/Zn superoxide dismutase 1. Expression of mutant Cu/Zn superoxide dismutase 1 in primary motor neurons altered the cellular localization of dynein, suggesting an inhibition of dynein/dynactin function. Thus, inhibition of dynein/dynactin function may have a role in motor neuron degeneration in amyotrophic lateral sclerosis.
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