“…In this report, we confirm that mitochondria from cells expressing mSOD1 have impaired ATP production, and this is associated with activation of the energy sensor AMPK. This observation is consistent with previous literature reporting dysregulation of various metabolic genes in mSOD1 models, including fatty acid synthase, fatty acid transporter (FAT/CD36), glucose transporter 4, p53, FOX03A, mTOR, and nitric oxide synthase, all of which are downstream targets of the AMPK pathway (Gonzalez de Aguilar et al, 2000;Martin, 2000;Lukas et al, 2006;Fergani et al, 2007;Lobsiger et al, 2007;Morimoto et al, 2007;Gonzalez de Aguilar et al, 2008;Martinez et al, 2008;Mojsilovic-Petrovic et al, 2009) (Lim and Kalb, unpublished observations). Furthermore, we show that AMPK activation has adverse effects on genetic models of motor neuron disease.…”