Modulation of the IGF1R-MTOR pathway attenuates motor neuron toxicity of human ALS SOD1^G93A astrocytes.

ABSTRACT: ALS (amyotrophic lateral sclerosis), the most common motor neuron disease, causes muscle denervation and rapidly fatal paralysis. While motor neurons are the most affected cells in ALS, studies on the pathophysiology of the disease have highlighted the importance of non-cell autonomous mechanisms, which implicate astrocytes and other glial cells. In ALS, subsets of reactive astrocytes lose their physiological functions and become toxic for motor neurons, thereby contributing to disease pathogenesis. Evidence of astrocyte contribution to disease pathogenesis are well established in cellular and animal models of familial ALS linked to mutant SOD1, where astrocytes promote motor neuron cell death. The mechanism underlying astrocytes reactivity in conditions of CNS injury have been shown to in

SUBMITTER: Granatiero V 

PROVIDER: S-EPMC8726657 | biostudies-literature | 2021 Dec

REPOSITORIES: biostudies-literature