Our finding of increased NLRP3 activation and reduced autophagy activation in human mC9-MG is in line with the existing literature reporting elevated levels of NLRP3 in astrocytes in sporadic ALS cases and suboptimal autophagy in the ventral horn of patients with ALS (48, 52, 53). Here, NLRP3 is linked to amyotrophic lateral sclerosis.