CASP3 and amyotrophic lateral sclerosis: On the other hand, the percentages of cleaved caspase 3-positive cells among the residual anterior horn cells in ALS patients with the E478G mutation and with the Q398X mutation of OPTN were 14.5% (9/62 cells) and 15.9% (7/44 cells), respectively, and those for cleaved caspase 8 were 14.3% (7/49 cells) and 16.7% (7/42 cells), respectively.