Post-mortem samples of ALS patients show the altered activity of ETC complexes (Bowling et al., 1993), while SOD1 overexpression in transgenic mice causes dysregulated ETC activity, increased ROS production, and diminished mitochondrial Ca2+-buffering (Mattiazzi et al., 2002; Brookes et al., 2004). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.