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The polymerized protein response (PPR) is activated by genetic variants that polymerize in the ER and is mediated by NFκBp50

Abstract In previous studies we have shown that accumulation of the polymerogenic variant ATZ that causes α1-antitrypsin deficiency (ATD) activates NFκB DNA binding. Here we discovered that this response, which we are naming the polymerized protein response (PPR), is characterized by NFκB p50 homodi...

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Principais autores: Admire Munanairi, David A. Rudnick, Jiansheng Huang, David H. Perlmutter
格式: Artigo
语言:Inglês
出版: Nature Portfolio 2026-04-01
丛编:Nature Communications
在线阅读:https://doi.org/10.1038/s41467-026-72369-w
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