Restrictive Lung Disease in the Cu/Zn Superoxide-Dismutase 1 G93A Amyotrophic Lateral Sclerosis Mouse Model

To our knowledge, the role of restrictive lung disease and the impact on the mechanics of the respiratory system have not been examined in the SOD1G93A mouse model. Because patients with ALS eventually succumb to respiratory failure as a result of restrictive lung disease and respiratory muscle weakness, we sought to characterize the pulmonary mechanics in SOD1G93A mice during disease progression. Many studies in ALS animal models use hind-limb weakness and survival to measure therapeutic success, without much emphasis on correction of the respiratory phenotype. Because respiratory failure is the primary cause of death in patients with ALS, we propose that future therapeutic studies using the SOD1G93A mouse model evaluate the impact of therapies on respiratory insufficiency and restrictive lung disease. *