PPARGC1A and amyotrophic lateral sclerosis: To investigate whether proteins involved in lipid biosynthesis and that are highlighted as potential modulators of ALS are expressed in Betz cells of ALS patients, and whether their presence is altered with respect to disease, we investigated the expression profiles of PPARG (Fig. 2b,c) and PPARGC1A (Fig. 2d,e) in the motor cortex of control cases (n = 12), as well as sALS patients with no known mutations (n = 8), and ALS patients with TDP43 pathology (n = 9).