Given that aberrant AMPK activation due to energy deficiency has been reported in degenerating neurons of several neurodegenerative diseases (including Alzheimer’s disease AD; Chen et al., 2009; Mairet-Coello et al., 2013; Ma et al., 2014; Chang et al., 2021), Huntington’s disease (HD; Ju et al., 2011, 2014), and amyotrophic lateral sclerosis (ALS; Lim et al., 2012; Liu et al., 2015a,b), a better understanding of the function and regulation of AMPK during neurodegeneration may provide potential targets for the development of new treatments for neurodegeneration diseases. Here, PRKAA2 is linked to amyotrophic lateral sclerosis.