“…Some cases of ALS have been described as a mitochondriopathy (Finsterer, 2002(Finsterer, , 2003 including a mitochondrial DNA www.intechopen.com mutation in the gene encoding subunit I of the mitochondrial respiratory chain complex IV (Comi et al, 1998). The electron transport chain proteins FAD synthetase, riboflavin kinase, cytochrome C1, and succinate dehydrogenase complex subunit B expression were significantly decreased in some ALS patients (Lin et al, 2009). In the mSOD1 mice or cell culture familial ALS model, complexes I, II and IV of the electron transport chain exhibit decreased enzyme activities, even at early stages of the disease Mattiazzi et al, 2002;Menzies et al, 2002a,b).…”