Despite paradoxical AMPK hyperactivation in ALS models, its phosphorylation levels are significantly reduced [107], with appropriate AMPK activation exerting neuroprotective effects through induction of autophagy to clear mutant proteins (reduced SOD1 aggregates) [108], phosphorylation of mTOR negative regulators to inhibit mTORC1 signaling and subsequent protein translation [109], and promotion of mitochondrial biogenesis and fatty acid metabolism to potentially slow disease progression [110]. Here, PRKAA1 is linked to amyotrophic lateral sclerosis.