Consequently, TNPO1 fails to counteract aberrant TDP-43 self-assembly, whereas the importin-α/β1 heterodimer which binds classical NLSs prevents seeded and unseeded fibril formation of wild-type TDP-43 and ALS-associated variant TDP-43Q331K in vitro [40]. This evidence concerns the gene TNPO1 and amyotrophic lateral sclerosis.