Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy.

"Declarations Ethics approval and consent to participateAll donors had provided written informed consent for the use of autopsy material and of clinical and genetic information for research purposes. Brain and spinal cord tissue was obtained postmortem following IRB review. All animal procedures were conducted in accordance with the guidelines set forth by the Institutional Animal Care and Use Committee (IACUC) at Mayo Clinic, and study protocols were reviewed and approved prior to performing the experimental procedures described. Consent for publicationAll authors read and approved the final manuscript. Competing interestsThe authors declare they have no financial competing interests. BK and WR are co-inventors on a patent relating to the content of the manuscript. Competing interests The authors declare they have no financial competing interests. BK and WR are co-inventors on a patent relating to the content of the manuscript."

"Funding This work was supported by grants from the Robert Packard Center for ALS Research, the Mayo Clinic Center for Biomedical Discovery, the BrightFocus foundation (A2021038S), the National Institutes of Health (NIH) R33NS110960, RF1AG068581, the US Department of Defense W81XWH-19–1-0193, a Mayo Clinic Ventures Innovation Loan to WR, and NIH R01AG077771 to WR and JS. DM was supported by the Audrey Lewis Young Investigator Award from the CureSMA foundation. DWD was supported by NIH awards P01NS084974 and P30AG062677, as well as the Rainwater Charitable Foundation and the Robert E. Jacoby Professorship. SJB was supported by NIH awards R01NS097542, R01NS113943, and P30AG072931. KZ was supported by NIH R01NS117461. CJD was supported by NIH R01NS105756, R21AG064940, R01NS127187 and the LiveLikeLou Center at the University of Pittsburgh Brain Institute and Target ALS Foundation. LP was supported by grants from the NIH (R35NS097273, U54NS123743, P01NS084974) and the Robert Packard Center for ALS Research at Johns Hopkins. This work was partially supported by NIH grants R01AG059871 and R21NS096647 to DER-L."