PRKAA1 and fragile X-associated tremor/ataxia syndrome: If these findings are confirmed in larger samples, they will suggest, in the light of earlier data (Herrero-Martın et al., 2009; He et al., 2014; Wu et al., 2014; Distelmaier et al., 2015; Hardie, 2015; Xu et al., 2017) that AMPK activity in PM carriers may be predominantly protective against stress-related cellular damage, thereby restraining the development of mitochondrial dysfunction as seen in FXTAS (Ross-Inta et al., 2010; Napoli et al., 2011).