Interestingly, although neurodegeneration and LF accumulation clearly correlate in most animal models, in this progranulin deficiency mouse model, it has been shown that deletion of the TMEM106B (Transmembrane protein 106B) gene normalizes lysosomal activity and rescues FTD-related behavioral abnormalities and retinal degeneration, without showing a decrease in LF accumulation (Klein et al., 2017); therefore, suggesting that neurodegeneration and lipofuscinogenesis are independent processes. This evidence concerns the gene TMEM106B and frontotemporal dementia.