Nuclear import pathway key to rescuing dominant progerin phenotypes

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


In this issue of Science Signaling, Larrieu et al. show that an acetyltransferase inhibitor that rescues many dominant nuclear phenotypes caused by progerin, a truncated form of lamin A, does so by releasing the specialized nuclear import receptor TNPO1 from sequestration by microtubules. This release enables TNPO1-dependent import of specific cargoes, including the nuclear pore protein Nup153 and the heterogeneous nuclear ribonucleoprotein hnRNPA1, thus restoring the functionality of nuclear pore complexes, rebalancing the nucleocytoplasmic Ran gradient, and normalizing gene expression.

Original languageEnglish (US)
Article number9448
JournalScience signaling
Issue number537
StatePublished - Jul 3 2018

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology


Dive into the research topics of 'Nuclear import pathway key to rescuing dominant progerin phenotypes'. Together they form a unique fingerprint.

Cite this