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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

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

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Chiarotto, Gabriela Bortolanca [1] ; Nardo, Giovanni [2] ; Trolese, Maria Chiara [2] ; Franca, Jr., Marcondes Cavalcante [3] ; Bendotti, Caterina [2] ; Rodrigues de Oliveira, Alexandre Leite [1]
Total Authors: 6
[1] Inst Biol Unicamp, Dept Struct & Funct Biol, BR-13083865 Campinas, SP - Brazil
[2] IRCCS Inst Pharmacol Res Mario Negri, Dept Neurosci, I-20156 Milan - Italy
[3] Fac Med Sci Unicamp, Dept Neurol, BR-13083887 Campinas, SP - Brazil
Total Affiliations: 3
Document type: Review article
Web of Science Citations: 1

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. (AU)

FAPESP's process: 14/06892-3 - Use of mesenchymal stem cells in the CNS/PNS interface: repair of proximal lesions
Grantee:Alexandre Leite Rodrigues de Oliveira
Support type: Research Projects - Thematic Grants
FAPESP's process: 13/16168-8 - Administration of riluzole, tempol and mesenchymal stem cells in the treatment of ALS in SOD1 G93A mice
Grantee:Gabriela Bortolança Chiarotto
Support type: Scholarships in Brazil - Doctorate
FAPESP's process: 17/02895-6 - Role of MHC Class I on ALS progression in 129Sv-G93A mice
Grantee:Gabriela Bortolança Chiarotto
Support type: Scholarships abroad - Research Internship - Doctorate