title: Major Histocompatibility Complex I Expression by Motor Neurons and Its Implication in Amyotrophic Lateral Sclerosis.
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.
authors.affiliation: Laboratory of Molecular Neurobiology, Department of Neuroscience, Mario Negri Institute for Pharmacological Research IRCCS , Milan , Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Nardo
authors.affiliation: Laboratory of Molecular Neurobiology, Department of Neuroscience, Mario Negri Institute for Pharmacological Research IRCCS , Milan , Italy.
authors.forename: Maria Chiara
authors.initials: MC
authors.lastname: Trolese
authors.affiliation: Laboratory of Molecular Neurobiology, Department of Neuroscience, Mario Negri Institute for Pharmacological Research IRCCS , Milan , Italy.
authors.forename: Caterina
authors.initials: C
authors.lastname: Bendotti
keywords: MHC class I; SOD1G93A mice; amyotrophic lateral sclerosis; motor neuron; neurodegeneration