Consistent with this are several observations: inhibiting TDP-43 localization to mitochondria or preventing fission blocks ALS-associated pathologies [277,291,292,293,294]; decreased MFF1 and 2 and increased DRP1 expression occur in motor neurons in a mouse model of ALS [295]; mitochondrial hyperfission has also been observed in the post-mortem neurons of patients diagnosed with ALS and other proteinopathies, as well as in cell cultures [284,285,291,292,294]. Here, TARDBP is linked to proteostasis deficiencies.