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Retrograde axonal transport and motor neuron disease
Author(s) -
Ström AnnaLena,
Gal Jozsef,
Shi Ping,
Kasarskis Edward J.,
Hayward Lawrence J.,
Zhu Haining
Publication year - 2008
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.2008.05393.x
Subject(s) - sod1 , axoplasmic transport , amyotrophic lateral sclerosis , dynein , motor neuron , neuroscience , microbiology and biotechnology , biology , superoxide dismutase , mutant , microtubule , biochemistry , medicine , pathology , disease , oxidative stress , gene , spinal cord
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.