PPARGC1A and amyotrophic lateral sclerosis: While no evidence currently exists for a direct interaction between PGC-1α and the proteins implicated in ALS pathophysiology, it is possible that the deficiency of PGC-1α and/or PGC-1α-dependent transcripts could contribute to the interneuron dysfunction in ALS, which is notably preceded by an accumulation of ROS and mitochondrial dysfunction [285].