TARDBP and frontotemporal dementia: Interestingly, low levels of TDP-43 have even been found to reside inside of mitochondria in human motor and cortical neurons; however, age-matched neurons from amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) patients expressed a significantly higher amount of mitochondrial TDP-43, reportedly altering their morphology and impairing mitochondrial function [33].