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UNRAVELING THE MECHANISMS INVOLVED IN MOTOR NEURON DEGENERATION IN ALS
Abstract
▪ Abstract Although Charcot described amyotrophic lateral sclerosis (ALS) more than 130 years ago, the mechanism underlying the characteristic selective degeneration and death of motor neurons in this common adult motor neuron disease has remained a mystery. There is no effective remedy for this progressive, fatal disorder. Modern genetics has now identified mutations in one gene [Cu/Zn superoxide dismutase (SOD1)] as a primary cause and implicated others [encoding neurofilaments, cytoplasmic dynein and its processivity factor dynactin, and vascular endothelial growth factor (VEGF)] as contributors to, or causes of, motor neuron diseases. These insights have enabled development of model systems to test hypotheses of disease mechanism and potential therapies. Along with errors in the handling of synaptic glutamate and the potential excitotoxic response this provokes, these model systems highlight the involvement of nonneuronal cells in disease progression and provide new therapeutic strategies.
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The ALS-linked G93A mutation of SOD1 allowed the establishment of a mouse model featuring MN degeneration in the spinal cord.
“The ALS-linked G93A mutation of SOD1 allowed the establishment of a mouse model, featuring MN degeneration in the SC4.”
From Retromer stabilization results in neuroprotection in a model of Amyotrophic Lateral Sclerosis (2020) · cites this paper as [4]
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Metadata
- Authors
- Bruijn, L. I., Miller, T. M., & Cleveland, D. W.
- Venue
- Annual Review of Neuroscience
- Publisher
- Annual Reviews
- Published
- 2004-07-21
- Volume
- 27
- Issue
- 1
- Pages
- 723-749
- ISSN
- 0147-006X
- 1545-4126
- Cited by
- 1,185
- References
- 3
- Language
- en
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