The most predominant responsible genes are the microtubule associated binding protein tau (MAPT, Hutton et al., 1998), which leads to a cascade of hyperphosphorylated tau; mutations in the gene encoding progranulin (PGRN), which causes FTD with ubiquitin and TDP43 inclusions, and the recently-identified expansion of the chromosome 9 open reading frame 72, which is defined by TDP43 proteinopathy (C9ORF72, DeJesus-Hernandez et al., 2011; Majounie et al., 2012). Here, GRN is linked to proteostasis deficiencies.