TARDBP and amyotrophic lateral sclerosis: Following induction of acute (exhaustive exercise-induced) or chronic (mtDNA mutation-induced) in vivo mitochondrial stress, mice deficient in Parkin or Pink1 accumulate mtDNA and present a type I interferon response in a STING-dependent way.210 The cytoplasmic and mitochondrial accumulation of TDP-43 is a hallmark in many cases of ALS and frontotemporal lobar degeneration (FTLD).