UCHL1 and amyotrophic lateral sclerosis: Transgenic mice expressing fluorescent labeled ubiquitin C-terminal hydrolase-L1 (UCHL1) in the CSMNs showed that these neurons are particularly sensitive to ER stress and to the lack of UCHL1 the ALS-associated gene, Alsin (Jara et al., 2015; Gautam et al., 2016).