Initially encompassing the inclusion body myopathy, Paget disease, frontotemporal dementia (IBMPFD) motor neuron disease spectrum, which includes VCP, HNRNPA2B1, and HNRNPA1 genes (MSP1, MSP2, and MSP3, respectively), MSP classification now incorporates the pathologies associated to SQSTM1 (MSP4), MATR3 (MSP5), TIA1, and eventually OPTN genes (28). This evidence concerns the gene HNRNPA2B1 and inclusion body myopathy with Paget disease of bone and frontotemporal dementia.