PPARGC1A and amyotrophic lateral sclerosis: To determine whether PGC-1α can protect against the motor neuron loss that accompanies the clinical symptoms of ALS, we counted the number of motor neurons in the lumbar spinal cord in age and gender matched WT, SOD1-G93A, PGC-1α and SOD1-G93A/PGC-1α mice at post-symptomatic stage (day 110) (Figure 3A).