The role of mutated SOD1 gene in synaptic stripping and MHC class I expression following nerve axotomy in ALS murine model


  • Roman M. Kassa University of Kentucky Medical Center, United States.
  • Roberta Bonafede University of Verona, Italy.
  • Federico Boschi University of Verona, Italy.
  • Manuela Malatesta University of Verona, Italy.
  • Raffaella Mariotti | University of Verona, Italy.


Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by motoneuron death. Several cellular pathways have been described to be involved in ALS pathogenesis; however, the involvement of presynaptic stripping and the related MHC class I molecules in mutant SOD1 motoneurons remains to be clarified. To this purpose, we here investigated, for the first time, the motoneurons behavior, di per seand after facial axonal injury, in terms of synaptic stripping and MHC class I expression in wild-type (Wt) mice and in a murine model of ALS, the SOD1(G93A) mice, at the presymptomatic and symptomatic stage of the disease. Concerning Wt animals, we found a reduction in synaptophysin immunoreactivity and an increase of MHC class I molecules in facial motoneurons after axotomy. In uninjured motoneurons of SOD1(G93A) mice, an altered presynaptic framework was evident, and this phenomenon increased during the disease course. The alteration in the presynaptic input is related to excitatory fibers. Moreover, after injury, a further decrease of excitatory input was not associated to an upregulation of MHC class I molecules in motoneuron soma. This study demonstrates, for the first time, that the presence of mutated SOD1 protein affects the MHC class I molecules expression, altering the presynaptic input in motoneurons. Nevertheless, a positive MHC class I immunolabeling was evident in glial cells around facial injured motoneurons, underlying an involvement of these cells in synaptic stripping. This study contributes to better understand the involvement of the mutated SOD1 protein in the vulnerability of motoneurons after damage.



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Original Papers
Amyotrophic lateral sclerosis, SOD1(G93A) mice, motoneurons, synaptophysin, major histocompatibility complex I, facial axotomy.
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How to Cite
Kassa, R. M., Bonafede, R., Boschi, F., Malatesta, M., & Mariotti, R. (2018). The role of mutated SOD1 gene in synaptic stripping and MHC class I expression following nerve axotomy in ALS murine model. European Journal of Histochemistry, 62(2).

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