The importance of mitophagy in the pathogenesis of neurodegenerative diseases is supported by the identified mutations of proteins involved in mitophagy—primarily PINK1 and parkin in Parkinson’s disease, optineurin and TBK1 in amyotrophic lateral sclerosis [17,18,19], and p62/SQSTM1 in frontotemporal dementia [20]. This evidence concerns the gene SQSTM1 and amyotrophic lateral sclerosis.