TY - JOUR
T1 - Nuclear import pathway key to rescuing dominant progerin phenotypes
AU - Wilson, Katherine L.
N1 - Funding Information:
thank K. S. Ullman for insightful comments on the manuscript. Funding: I acknowledge funding from the Progeria Research Foundation, and the Johns Hopkins University Claude D. Pepper Older Americans Independence Center of the National Institute on Aging (grant no. P30AG021334). Competing interests: The author declares that she has no competing interests.
Publisher Copyright:
Copyright © 2018 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works
PY - 2018/7/3
Y1 - 2018/7/3
N2 - 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.
AB - 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.
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U2 - 10.1126/scisignal.aat9448
DO - 10.1126/scisignal.aat9448
M3 - Article
C2 - 29970604
AN - SCOPUS:85049412462
SN - 1945-0877
VL - 11
JO - Science signaling
JF - Science signaling
IS - 537
M1 - 9448
ER -