Mutations affecting several RBPs, including FUS, TDP-43, hnRNPA1, hnRNPA2B1, matrin-3, and TIA1, are linked to amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), multisystem proteinopathy (MSP), and hereditary inclusion body myopathy (hIBM) phenotypes (8, 9). The gene discussed is HNRNPA2B1; the disease is frontotemporal dementia.