The emerging role of the major histocompatibility complex class I in amyotrophic lateral sclerosis

Gabriela Bortolança Chiarotto, Giovanni Nardo, Maria Chiara Trolese, Marcondes Cavalcante França, Caterina Bendotti, Alexandre Leite Rodrigues De Oliveira

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Article number2298
JournalInternational Journal of Molecular Sciences
Issue number11
Publication statusPublished - Nov 1 2017


  • Amyotrophic lateral sclerosis
  • Glial cells
  • Major histocompatibility complex I
  • Microglia
  • Neuroprotection

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry


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