… , nuclear import of FUS is impaired when the nuclear … To gain insight into how mutant FUS
disrupts NCT, we explored … implicate impaired NCT in FUS-mediated ALS and support the …

… , nuclear import of FUS is impaired when the nuclear … To gain insight into how mutant FUS
disrupts NCT, we explored … implicate impaired NCT in FUS-mediated ALS and support the …

… FUSEC has a unique N-terminus sequence but shares its C-terminus with canonical FUS.
For FUS, the most abundant ALS mutations are … FUS without a nuclear localization signal …

… β-mediated nuclear transport which therefore perturbs nuclear … PDI to restore nuclear import
in cells expressing mutant FUS. … Disruption of nucleocytoplasmic transport is also induced by …

… disruption of RBP homeostasis plays a critical role in FUS-… interactions with the nuclear
import receptor Transportin-1 [6]. … To evaluate neurodegeneration mediated by RBP knockdown, …

… that recognize nuclear localization signals (NLS) in their cargo and mediate … of ALS- and
FTD-associated FUS mutations are located in its NLS and disrupt its normal nuclear localization …

… -alanine are also reported to be associated with ALS [17,18]. … of pathogenic mutation affecting
the nuclear import of FUS. In … The mutation disrupts the spatial arrangement of the binding …

… and those mediating nuclear import are known as importins. … are sufficient to disturb
nucleocytoplasmic transport. First, … In addition, many of the ALS-associated mutations in FUS are …

… transcription, the splicing and transport of mRNA, post-… Elevated expression levels and
the strong nuclear localization of … of NPM-hMLF1 may disturb the regulation of p53 stability, …

… points towards FUS having a vital cytoplasmic role for mRNA transport within axons and …
of ALS-FUS 22,33 . Therefore, we sought to establish whether mutant FUS disrupted the …