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.

中文翻译：

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核输入途径是拯救显性早老蛋白表型的关键。

在本期《科学信号》中，Larrieu等人表明，乙酰转移酶抑制剂可以挽救由progerin（一种截短形式的lamin A）引起的许多显性核表型，通过释放专用的核输入受体TNPO1使其从微管隔离中释放出来。此版本使特定货物的TNPO1依赖进口，包括核孔蛋白Nup153和异质核核糖核蛋白hnRNPA1，从而恢复了核孔复合物的功能，重新平衡了核质Ran梯度，并使基因表达正常化。