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Review
. 2021 Mar;82(3):155-161.
doi: 10.1016/j.humimm.2021.01.017. Epub 2021 Feb 12.

The role of immune-mediated alterations and disorders in ALS disease

João Rodrigues Lima-Junior  1 , David Sulzer  2 , Cecilia S Lindestam Arlehamn  1 , Alessandro Sette  3
Affiliations
Review

The role of immune-mediated alterations and disorders in ALS disease

João Rodrigues Lima-Junior et al. Hum Immunol. 2021 Mar.

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that leads to neuronal death in the brain and spinal cord. Over the last decades, evidence has emerged regarding the functional diversity of astrocytes, microglia, and T cells in the central nervous system (CNS), and the role of neuroinflammation in ALS. In this review, we summarize current knowledge regarding neuroinflammation in ALS, both at the level of specific molecular pathways and potential cellular pathways as well as outline questions about the immune mechanisms involved in ALS pathogenesis.

Keywords: ALS; Autoimmunity; Glial cells; Neuroinflammation.

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Conflict of interest statement

Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Figure 1.
Figure 1.. CD4+ T cell reactivity in ALS disease.
Misfolded protein aggregates are taken up by dendritic cells and reach the draining lymph nodes, where proteins can be presented as self-peptides through MHC class II for recognition by CD4+ T cells. After priming, antigen-specific T cells migrate to the CNS. Upon activation, microglia upregulate MHC class II and act as antigen-presenting cells to restimulate CNS-infiltrating T cells, promoting neuroinflammation.
See this image and copyright information in PMC

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