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Int. J. Mol. Sci. 2018, 19(2), 631; https://doi.org/10.3390/ijms19020631

Temporospatial Analysis and New Players in the Immunology of Amyotrophic Lateral Sclerosis

1
Anatomy and Cell Biology Department, Indiana University School of Medicine, Indianapolis, IN 46202, USA
2
Richard L. Roudebush Veterans Affairs Medical Center, Indianapolis, IN 46202, USA
3
Department of Cancer Biology and Genetics, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA
4
Department of Biomedical and Applied Sciences, Indiana University School of Dentistry, Indianapolis, IN 46202, USA
*
Author to whom correspondence should be addressed.
Received: 1 February 2018 / Revised: 18 February 2018 / Accepted: 21 February 2018 / Published: 23 February 2018
(This article belongs to the Collection Neuroprotective Strategies)
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Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive loss of lower and upper motor neurons (MN) leading to muscle weakness, paralysis and eventually death. Although a highly varied etiology results in ALS, it broadly manifests itself as sporadic and familial forms that have evident similarities in clinical symptoms and disease progression. There is a tremendous amount of knowledge on molecular mechanisms leading to loss of MNs and neuromuscular junctions (NMJ) as major determinants of disease onset, severity and progression in ALS. Specifically, two main opposing hypotheses, the dying forward and dying back phenomena, exist to account for NMJ denervation. The former hypothesis proposes that the earliest degeneration occurs at the central MNs and proceeds to the NMJ, whereas in the latter, the peripheral NMJ is the site of precipitating degeneration progressing backwards to the MN cell body. A large body of literature strongly indicates a role for the immune system in disease onset and progression via regulatory involvement at the level of both the central and peripheral nervous systems (CNS and PNS). In this review, we discuss the earliest reported immune responses with an emphasis on newly identified immune players in mutant superoxide dismutase 1 (mSOD1) transgenic mice, the gold standard mouse model for ALS. View Full-Text
Keywords: amyotrophic lateral sclerosis (ALS); immune response; neuroimmunology; motor neuron disease amyotrophic lateral sclerosis (ALS); immune response; neuroimmunology; motor neuron disease
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Iyer, A.K.; Jones, K.J.; Sanders, V.M.; Walker, C.L. Temporospatial Analysis and New Players in the Immunology of Amyotrophic Lateral Sclerosis. Int. J. Mol. Sci. 2018, 19, 631.

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