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Neurobiol Dis. 2015 May;77:1-12. doi: 10.1016/j.nbd.2015.02.017. Epub 2015 Feb 26.

The dual roles of immunity in ALS: injury overrides protection.

Author information

1
Department of Neurology, University of Michigan, 109 Zina Pitcher Place, 5017 AAT-BSRB, Ann Arbor, MI, USA. Electronic address: murdockb@umich.edu.
2
Department of Neurology, University of Michigan, 109 Zina Pitcher Place, 5017 AAT-BSRB, Ann Arbor, MI, USA. Electronic address: dianeben@med.umich.edu.
3
Department of Neurology, University of Michigan, 109 Zina Pitcher Place, 4013 AAT-BSRB, Ann Arbor, MI, USA. Electronic address: bmsegal@med.umich.edu.
4
Department of Neurology, University of Michigan, 109 Zina Pitcher Place, 5017 AAT-BSRB, Ann Arbor, MI, USA. Electronic address: efeldman@umich.edu.

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease affecting motor neurons. Disease progression is accompanied by a multi-phased immune response, and recent studies indicate that the immune system is not simply a bystander during disease, but plays an active role in shaping ALS pathology. The role of the immune system during ALS progression is highly complex, however, as it has been found to have a role in both enhancing neurodegeneration as well as protecting the central nervous system. Previous reports have established that the immune response can therefore be separated into two distinct phases: a protective Type 2 response followed by a neurotoxic Type 1 response. This review will address the two phases of the immune response in ALS and describe their roles during disease progression. More importantly, it will also examine the likely sources of immune polarization that are responsible for shifting immunity from the protective T2 phase to the neurotoxic T1 phase.

KEYWORDS:

Amyotrophic lateral sclerosis; Central nervous system; Debris clearance; Immune response; Monocytes; Mutant SOD1; Repair; Toll-like receptors

PMID:
25726748
DOI:
10.1016/j.nbd.2015.02.017
[Indexed for MEDLINE]

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