Finally, we evaluated the impact of the three compounds on accumulation of subunit c of the mitochondrial ATPase, which is the main component of the lysosomal storage material in CLN3 disease [53,54] and which we have previously shown progressively accumulates in CbCln3∆ex7/8/∆ex7/8 cells upon aging [17]. The gene discussed is ATP5F1E; the disease is CLN3 disease.