ATG4B and amyotrophic lateral sclerosis: Indeed, CEs within the RNA targets of TDP-43, such as G protein signaling modulator (GPSM2), autophagy-related 4B cysteine peptidase (ATG4B), and UNC13A, have been detected in the frontal cortex of patients with sporadic ALS and FTLD due to TDP-43 proteinopathy (Brown et al., 2022; Ling et al., 2015; Ma et al., 2022), suggesting that the splicing activity of TDP-43 is impaired in these patients.