A similar effect can be observed in the IBMPFD (inclusion body myopathy with Paget’s disease of the bone and frontotemporal dementia) mutant L198W (Fig. 1C), indicating the activation of ATPase activity at the D1-domain by the D1-D2 linker is not affected by the introduction of the pathogenic mutation. The gene discussed is DNAH8; the disease is frontotemporal dementia.