Neuron‐specific knockout of the essential autophagy genes ATG7, ATG5, and RB1CC1 (FIP200) in mice causes neurodegeneration, progressive deficits in motor function, including abnormal limb‐clasping reflexes (as also observed in ALS‐SOD1 mice) and a reduction in coordinated movement, that are accompanied by the accumulation of cytoplasmic inclusion bodies in neurons (Hara et al, 2006; Komatsu et al, 2006; Liang et al, 2010). Here, RB1CC1 is linked to amyotrophic lateral sclerosis.