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Int. J. Mol. Sci. 2017, 18(11), 2298; https://doi.org/10.3390/ijms18112298

The Emerging Role of the Major Histocompatibility Complex Class I in Amyotrophic Lateral Sclerosis

1
Department of Structural and Functional Biology, Institute of Biology—Unicamp, 13083-865 Campinas, Brazil
2
Department of Neuroscience, IRCCS—Institute of Pharmacological Research Mario Negri, 20156 Milano, Italy
3
Departament of Neurology, Faculty of Medical Sciences—Unicamp, 13083-887 Campinas, Brazil
These authors contributed equally to this work.
*
Authors to whom correspondence should be addressed.
Received: 21 September 2017 / Revised: 16 October 2017 / Accepted: 26 October 2017 / Published: 1 November 2017
(This article belongs to the Special Issue Major Histocompatibility Complex)
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Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting upper and lower motoneurons (MNs). The etiology of the disease is still unknown for most patients with sporadic ALS, while in 5–10% of the familial cases, several gene mutations have been linked to the disease. Mutations in the gene encoding Cu, Zn superoxide dismutase (SOD1), reproducing in animal models a pathological scenario similar to that found in ALS patients, have allowed for the identification of mechanisms relevant to the ALS pathogenesis. Among them, neuroinflammation mediated by glial cells and systemic immune activation play a key role in the progression of the disease, through mechanisms that can be either neuroprotective or neurodetrimental depending on the type of cells and the MN compartment involved. In this review, we will examine and discuss the involvement of major histocompatibility complex class I (MHCI) in ALS concerning its function in the adaptive immunity and its role in modulating the neural plasticity in the central and peripheral nervous system. The evidence indicates that the overexpression of MHCI into MNs protect them from astrocytes’ toxicity in the central nervous system (CNS) and promote the removal of degenerating motor axons accelerating collateral reinnervation of muscles. View Full-Text
Keywords: major histocompatibility complex I; amyotrophic lateral sclerosis; glial cells; microglia; neuroprotection major histocompatibility complex I; amyotrophic lateral sclerosis; glial cells; microglia; neuroprotection
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Chiarotto, G.B.; Nardo, G.; Trolese, M.C.; França Jr., M.C.; Bendotti, C.; Rodrigues de Oliveira, A.L. The Emerging Role of the Major Histocompatibility Complex Class I in Amyotrophic Lateral Sclerosis. Int. J. Mol. Sci. 2017, 18, 2298.

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