In mouse models, it was found that CysC has neuroprotective activity against ALS SOD-mediated toxicity, exogenous addition of CysC protects neuronal cells, including primary cultured motor neurons, depending on the coordinated activation of two different pathways: autophagy induction via AMPK-mTOR pathway and cathepsin B inhibition (Watanabe et al., 2014), the former can promote the degradation of misfolded or unfolded proteins and prevent the accumulation of abnormal mutant proteins, while the latter is closely related to the degeneration of motor neurons (Kikuchi et al., 2003). This evidence concerns the gene CTSB and amyotrophic lateral sclerosis.