Similar to PPARG, the presence of PPARGC1A protein was evident in the Betz cells of control cases (n = 12), albeit at low levels (Fig. 2e), and was increased in the Betz cells of sALS patients and ALS patients with TDP43 pathology (average percentage of Betz cells with increased PPARGC1A expression: control: 27 ± 6; sALS: 82 ± 3; TDP43: 81 ± 4; Fig. 2e). This evidence concerns the gene PPARGC1A and amyotrophic lateral sclerosis.