PPARGC1A and amyotrophic lateral sclerosis: Underscoring the relevancy of PGC-1α to the pathogenesis of ALS, PGC-1α expression has been shown to be reduced in the muscles [17,18], motor cortex, and spinal cord motor neurons of ALS patients17, as well as in muscle [17,19] and spinal cord of the SOD1G93A mouse model of ALS [17].