Maria Teresa Golia, Roberto Frigerio, Susanna Pucci, Francesca Sironi, Cassandra Margotta, Laura Pasetto, Camilla Testori, Elena Berrone, Francesco Ingravalle, Marcella Chiari, Alessandro Gori, Roberto Duchi, Andrea Perota, Luca Bergamaschi, Antonio D'Angelo, Giulia Cagnotti, Cesare Galli, Cristiano Corona, Valentina Bonetto, Caterina Bendotti, Marina Cretich, Sara Francesca Colombo, Claudia Verderio
SOD1 gene is associated with progressive motor neuron degeneration in the familiar forms of amyotrophic lateral sclerosis. Although studies on mutant human SOD1 transgenic rodent models have provided important insights into disease pathogenesis, they have not led to the discovery of early biomarkers or effective therapies in human disease. The recent generation of a transgenic swine model expressing the human pathological hSOD1G93A gene, which recapitulates the course of human disease, represents an interesting tool for the identification of early disease mechanisms and diagnostic biomarkers...
February 6, 2024: Experimental Neurology