Revisiting the role of the innate immune complement system in ALS

Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing motor neuron disease without effective treatment. Although the precise mechanisms leading to ALS are yet to be determined, there is now increasing evidence implicating components of the innate immune complement system in the onse...

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Bibliographic Details
Published in:Neurobiology of disease 2019-07, Vol.127, p.223-232
Main Authors: Parker, Sandra E., Hanton, Angela M., Stefanou, Stephen N., Noakes, Peter G., Woodruff, Trent M., Lee, John D.
Format: Article
Language:English
Subjects:
Amyotrophic Lateral Sclerosis - immunology
Amyotrophic Lateral Sclerosis - metabolism
Animals
Complement System Proteins - metabolism
Humans
Immunity, Innate - physiology
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Summary:Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing motor neuron disease without effective treatment. Although the precise mechanisms leading to ALS are yet to be determined, there is now increasing evidence implicating components of the innate immune complement system in the onset and progression of its motor phenotypes. This review will survey the clinical and experimental evidence for the role of the complement system in driving neuroinflammation and contributing to ALS disease progression. Specifically, it will explore findings regarding the different complement activation pathways involved in ALS, with a focus on the terminal pathway. It will also examine potential future research directions for complement in ALS, highlighting the targeting of specific molecular components of the system.
ISSN:0969-9961
1095-953X
DOI:10.1016/j.nbd.2019.03.003