Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder
characterized by the progressive degeneration of both upper and lower motor neurons
leading to paralysis and finally to death. Non-neuronal cells, including glial cells, have
been shown to actively participate in the physiopathological process occurring in ALS.
Experiments using chimeric mice expressing ALS-linked mutations suggest that
neighboring non-neuronal cells modulate disease phenotype. In this review, recent
findings involving the role of astrocytes, microglia and of other non-neuronal cells will
be discussed. The study of motor neuron microenvironment could lead to a better
understanding of the physiopathology of ALS to find new pathways to slow down
motor neuron degeneration.
Keywords: Amyotrophic lateral sclerosis, ALS, amyotrophia, animal models,
astrocytes, CNS, glial cells, microglia, motor neuron, motor neuron disease,
neurodegeneration, neuroinflammation, neuroprotection, neurotoxicity, non-cell
autonomous, non-neuronal cells, progressive paralysis, SOD1, spinal cord,
transgenic mice.
