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Article

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

Nature communications - 27 Apr 2026

Munanairi Admire, Rudnick David A, Huang Jiansheng, Perlmutter David H

Abstract excerpt

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 homodimers, distinct from the p50/p65 heterodimers in the canonical NFκB inflammatory response and the unfolded protein response...

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Share to alpha 1-Antitrypsin
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 42045190
DOI: 10.1038/s41467-026-72369-w
PMCID: PMC13332235

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 8 Sept 2026.

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