NLRP3 and amyotrophic lateral sclerosis: Transactive response DNA-binding protein-43 (TDP-43) is also implicated in ALS pathology, as it forms a major component of intraneuronal aggregates in most ALS patients (Prasad et al., 2019), as well as triggering mtDNA release, acting as a trigger for the NLRP3 inflammasome (Yu et al., 2020).