Previous reports have implicated LGMN as the key protease involved in the pathogenesis of AD, frontotemporal dementia and Parkinson disease due to its ability to cleave proteins accumulating in pathological inclusions typical of these diseases, such as Tau, α-synuclein and TDP-43 [13, 26, 28, 29] among many other substrates [8]. This evidence concerns the gene MAPT and frontotemporal dementia.