The pathological TDP-43 modification processes in TDP-43 proteinopathy may include cysteine oxidation [23,24] and acetylation [25] of TDP-43, nuclear to cytoplasmic mislocalization of aberrant RNA-binding proteins, increased aggregation, perturbed stress granule dynamics, and cell injury caused by RNA-binding protein localization to mitochondria [26]. Here, TARDBP is linked to proteostasis deficiencies.