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Article

Defining the mechanism of polymerization in the serpinopathies.

Proceedings of the National Academy of Sciences of the United States of America - 5 Oct 2010

Ekeowa Ugo I, Freeke Joanna, Miranda Elena, Gooptu Bibek, Bush Matthew F, Pérez Juan, Teckman Jeff, Robinson Carol V, Lomas David A

Abstract excerpt

The serpinopathies result from the ordered polymerization of mutants of members of the serine proteinase inhibitor (serpin) superfamily. These polymers are retained within the cell of synthesis where they cause a toxic gain of function. The serpinopathies are exemplified by inclusions that form with the common severe Z mutant of α(1)-antitrypsin that are associated with liver cirrhosis. There is considerable...

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Identifiers

PMID: 20855577
DOI: 10.1073/pnas.1004785107
PMCID: PMC2951428

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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