Pathogenic missense variants in RBPs such as TDP-434,5, hnRNPA16–9, hnRNPA2/B16,10, hnRNPDL11–14, FUS15,16, and TIA117,18 cause a spectrum of diseases with pleomorphic phenotypic manifestations including amyotrophic lateral sclerosis (ALS)/motor neuron disease, frontotemporal dementia (FTD), inclusion body myopathy (IBM), distal myopathy, and Paget’s disease of bone (PDB). The gene discussed is HNRNPA2B1; the disease is frontotemporal dementia.