TY - JOUR
T1 - Retrograde axonal transport and motor neuron disease
AU - Ström, Anna Lena
AU - Gal, Jozsef
AU - Shi, Ping
AU - Kasarskis, Edward J.
AU - Hayward, Lawrence J.
AU - Zhu, Haining
PY - 2008/7
Y1 - 2008/7
N2 - 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-medi- ated 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.
AB - 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-medi- ated 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.
KW - Amyotrophic lateral sclerosis
KW - Axonal transport
KW - Dynein
KW - Motor neuron
KW - Superoxide dismutase
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U2 - 10.1111/j.1471-4159.2008.05393.x
DO - 10.1111/j.1471-4159.2008.05393.x
M3 - Review article
C2 - 18384644
AN - SCOPUS:50849083264
SN - 0022-3042
VL - 106
SP - 495
EP - 505
JO - Journal of Neurochemistry
JF - Journal of Neurochemistry
IS - 2
ER -