Hence, the identified ELAVL3-pathologies [17] and our observation that NOVA1 exhibited increased insolubility in this iPSC-MN model of early stage ALS and higher cytoplasmic levels in postmortem MNs that do not yet show TDP-43 pathology strengthen the conclusion that abnormal RBPs alterations occur early in the pathogenesis of the disease. Here, ELAVL3 is linked to amyotrophic lateral sclerosis.