As detailed above, both wild-type and FTD/ALS mutants of TDP43 disrupt the VAPB-PTPIP51 interaction, ER-mitochondria contacts, IP3 receptor delivery of Ca2+ to mitochondria and activate GSK3β to similar extents, and induce indistinguishable disease phenotypes in transgenic mice [47, 52]. Here, ITPR1 is linked to frontotemporal dementia.