PDH activity is important formaintaining energy homeostasis in ALS, as knockdown of pyruvate dehydrogenasekinase 2 (PDK2), that inhibits PDH, is able to support mitochondrial metabolismin SOD1-G93A rat model, by ameliorating the interplay between astrocytes andmotor neurons [68].In light of this, PDH expression could represent a hub for the regulation ofenergy metabolism in ALS, also in peripheral tissues. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.