In this case, the two cutting-edge studies published in the year 2006 showed that haploinsufficiency and null mutations of the PGRN gene were recognized as a determinant of diverse familiar forms of frontotemporal lobar degeneration (FTLD), sharing the neuropathology of neural intracellular inclusions, proteinopathy, and the trigger landmark research aimed at explaining PGRN function in the CNS [9, 10]. This evidence concerns the gene GRN and proteostasis deficiencies.