Pathological Sequelae Associated with Skeletal Muscle Atrophy and Histopathology in G93A*SOD1 Mice

Amyotrophic lateral sclerosis (ALS) is a complex systemic disease that primarily involves motor neuron dysfunction and skeletal muscle atrophy. One commonly used mouse model to study ALS was generated by transgenic expression of mutant form human superoxide dismutase 1 (SOD1) gene harboring single amino acid substitution glycine alanine at codon 93 (G93A*SOD1). Although mutant-SOD1 ubiquitously expressed in G93A*SOD1 mice, detailed analysis the pattern protein resultant pathology were never performed. Using different muscles isolated from we extensively characterized pathological sequelae histological, molecular, ultrastructural, biochemical alterations. Muscle atrophy mice associated with increased differential across myofibers MuRF1 level. In addition, high collagen deposition myopathic changes sections accompanied reduced strength mice. Furthermore, all showed altered levels signaling pathways, including inflammation, mitochondrial membrane transport, lipid uptake, antioxidant enzymes. found fraction which vacuolized abnormal mitochondria, OXPHOS PDH levels, defects respiration. Overall, reported observed resulting whole-body expression.