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Human SOD1-G93A specific distribution evidenced in murine brain of a transgenic model for Amyotrophic Lateral Sclerosis by MALDI Imaging Mass Spectrometry


Acquadro Elena; Caron Ilaria; Tortarolo Massimo; Bucci Enrico; Bendotti Caterina; Corpillo Davide

J. Proteome Res., Just Accepted Manuscript


Amyotrophic Lateral Sclerosis (ALS) is a progressive, fatal neurodegenerative disease caused by the degeneration of motor neurons. The transgenic mouse model carrying the human SOD1G93A mutant gene (hSOD1G93A mouse) represents one of the most reliable and widely used model of this pathology. In the present work, the innovative technique of matrix-assisted laser desorption/ionization (MALDI) imaging mass spectrometry (IMS) was applied in the study of pathological alterations at the level of small brain regions such as facial and trigeminal nuclei, which in rodents are extremely small and would be difficult to analyze with classical proteomics approaches. Comparing slices from three mice groups (transgenic hSOD1G93A, transgenic hSOD1WT and non-transgenic, Ntg), this technique allowed to evidence the accumulation of hSOD1G93A in the facial and trigeminal nuclei, where it generates aggregates. This phenomenon is likely to be correlated to the degeneration observed in these regions. Moreover, a statistical analysis allowed to highlight other proteins as differentially expressed among the three mice groups analyzed. Some of them were identified by Reverse Phase-HPLC fractionation of extracted proteins and mass spectrometric analysis before and after trypsin digestion. In particular, the 40S ribosomal protein S19 (RPS19) resulted upregulated in the parenkyma and reactive glial cells in facial nuclei of hSOD1G93A mice when compared to transgenic hSOD1WT and non-transgenic ones.

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