HTT and Huntington disease: Indeed, AMPK activation should lead to the activation of autophagy and mitophagy, but this effect is likely compromised in the HD context, as we implied by the accumulation of mutant huntingtin, LC3-II, and the appearance of altered mitochondria in double mutant HD; Scn4a skeletal muscles.10 Therefore, the possible benefits of incrementing mitochondrial function in HD pathogenesis may be observed only when they are accompanied by a continuous supply of energy in an environment where those mitochondria might not be damaged by the presence of mutant huntingtin.