Major histocompatibility complex I expression by motor neurons and its implication in amyotrophic lateral sclerosis

Giovanni Nardo, Maria Chiara Trolese, Caterina Bendotti

Research output: Contribution to journalReview article

Abstract

Neuronal expression of major histocompatibility complex I (MHCI)-related molecules in adults and during CNS diseases is involved in the synaptic plasticity and axonal regeneration with mechanisms either dependent or independent of their immune functions. Motor neurons are highly responsive in triggering the expression of MHCI molecules during normal aging or following insults and diseases, and this has implications in the synaptic controls, axonal regeneration, and neuromuscular junction stability of these neurons. We recently reported that MHCI and immunoproteasome are strongly activated in spinal motor neurons and their peripheral motor axon in a mouse model of familial amyotrophic lateral sclerosis (ALS) during the course of the disease. This response was prominent in ALS mice with slower disease progression in which the axonal structure and function was better preserved than in fast-progressing mice. This review summarizes and discusses our observations in the light of knowledge about the possible role of MHCI in motor neurons providing additional insight into the pathophysiology of ALS.

Original languageEnglish
Article number89
JournalFrontiers in Neurology
Volume7
Issue numberJUN
DOIs
Publication statusPublished - 2016

Keywords

  • Amyotrophic lateral sclerosis
  • MHC class I
  • Motor neuron
  • Neurodegeneration
  • SOD1 mice

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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