Interestingly, not only complete loss but also haploinsufficiency of PGRN has been shown to increase tau pathology in P301L (JNPL3) and P301S (PS19) mouse models of tauopathy [49, 185], although tau-mediated neurodegeneration was paradoxically attenuated in PGRN-deficient PS19 tauopathy mice [49]. The gene discussed is GRN; the disease is tauopathy.