Characterization of early pathogenesis in the SOD1G93A mouse model of ALS: part I, background and methods (pages 335–350)
Sharon Vinsant, Carol Mansfield, Ramon Jimenez-Moreno, Victoria Del Gaizo Moore, Masaaki Yoshikawa, Thomas G. Hampton, David Prevette, James Caress, Ronald W. Oppenheim and Carol Milligan
Article first published online: 11 JUN 2013 | DOI: 10.1002/brb3.143
Here, we describe rationale and methods for our extensive analysis that included ultrastructural examination of central and peripheral components of the neuromuscular system in the superoxide dismutase 1 (SOD1G93A) mouse and correlated these alterations with early muscle denervation, motor dysfunction, and motoneuron death. We also provide a review of published work describing pathology in the SOD1 mouse model. In the companion paper we present the experimental results, discuss our interpretation of these findings, and summarize the chronology of pathogenesis. The significance of this work is that we have examined early pathology in both spinal cord and peripheral neuromuscular system and demonstrate that a thorough characterization of animal models throughout the life span is critical to design preclinical trials that will produce meaningful results.