We subsequently discovered substantial increases in STAU1 in multiple cell and animal models of human neurodegenerative diseases, including those carrying mutations in presenilin1, microtubule-associated protein tau, huntingtin, TAR DNA-binding protein-43 gene (TARDBP), or C9orf72–SMCR8 complex subunit (C9orf72) [1], as well as stroke and myotonic dystrophy [1, 3, 12]. Here, TARDBP is linked to myotonic dystrophy.