Sig-1R is encoded by the SIGMAR1 gene, and several mutations on its gene have been identified that lead to different types of MND, such as a severe, juvenile-onset form of ALS (ALS16) [6], development of frontotemporal dementia (FTD)-ALS [7], and some familial cases of distal hereditary motor neuropathies (dHMNs) [8,9]. The gene discussed is SIGMAR1; the disease is frontotemporal dementia.