These results suggest that excitotoxicity drives a die-forward mechanism of motor neuron death from the cell body outward to distal neuronal compartments.
Amyotrophic lateral sclerosis (ALS) is caused by motor neuron death.
Loss of calcium homeostasis is another factor which may contribute to motor neuron death in ALS cases (Bruijn et al., 2004).
Investigation of the role that mitochondria play in motor neuron death in ALS has taken place in a range of experimental systems.
The final process of motor neuron death encompasses many features of apoptosis, but it is clear that this alone cannot account for all features of motor neuron loss and that aspects of a necrosis-apoptosis continuum are at play.
These studies strongly emphasize a microglial contribution to the propagation of motor neuron death.
Since all animals remained in the study until end-stage of disease, typically a result of cumulative motor neuron death, analysis of motor neuron death in our study is likely to be uninformative.
Development of an effective treatment is complicated by the diffuse nature of motor neuron death; cell therapy may be a promising new treatment for ALS.
Other than its function in restricting neurite outgrowth, NgR also has an apparent role in preventing NGF-stimulated p75NTR-dependent motor neuron death as recently shown [41].
Recent work in mSOD1 mice showed that motor neuron death is not cell autonomous and involves defects in other cell types than neurons [3], [4].
Motor neuron death in amyotrophic lateral sclerosis (ALS) is considered a "non-cell autonomous" process, with astrocytes playing a critical role in disease progression.
