Journal
JOURNAL OF NEUROCHEMISTRY
Volume 106, Issue 2, Pages 495-505Publisher
WILEY
DOI: 10.1111/j.1471-4159.2008.05393.x
Keywords
amyotrophic lateral sclerosis; axonal transport; dynein; motor neuron; superoxide dismutase
Categories
Funding
- NINDS NIH HHS [R01 NS045087, R01 NS049126, R01-NS44170, R01-NS045087, R01 NS044170, R01-NS49126] Funding Source: Medline
Ask authors/readers for more resources
Transport of material between extensive neuronal processes and the cell body is crucial for neuronal function and survival. Growing evidence shows that deficits in axonal transport contribute to the pathogenesis of multiple neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Here we review recent data indicating that defects in dynein-mediated retrograde axonal transport are involved in ALS etiology. We discuss how mutant copper-zinc superoxide dismutase (SOD1) and an aberrant interaction between mutant SOD1 and dynein could perturb retrograde transport of neurotrophic factors and mitochondria. A possible contribution of axonal transport to the aggregation and degradation processes of mutant SOD1 is also reviewed. We further consider how the interference with axonal transport and protein turnover by mutant SOD1 could influence the function and viability of motor neurons in ALS.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available