It is noteworthy that ALS-linked mutations in OPTN, SQSTM1, and TBK1 can interfere with selective degradation of mitochondria via autophagy (mitophagy) and lead to inefficient turnover of damaged mitochondria, which may contribute to the progression of the neurodegenerative disease [24,25]. The gene discussed is OPTN; the disease is amyotrophic lateral sclerosis.