We further tested VCP function in luciferase reporter assays by measuring BMP-induced luciferase reporter (BRE-Luc) activity in mouse pluripotent mesenchymal KS483 cells [17] overexpressing either two missense mutations, VCPR95G and VCPR155H, responsible for Inclusion body myopathy associated with Paget disease of the bone and frontotemporal dementia (IBMPFD), or wild-type VCP [28], [29]. Here, VCP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.