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Article

A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activity.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Jun 2015

Ordóñez Adriana, Pérez Juan, Tan Lu, Dickens Jennifer A, Motamedi-Shad Neda, Irving James A, Haq Imran, Ekeowa Ugo, Marciniak Stefan J, Miranda Elena, Lomas David A

Abstract excerpt

Mutant Z α1-antitrypsin (E342K) accumulates as polymers within the endoplasmic reticulum (ER) of hepatocytes predisposing to liver disease, whereas low levels of circulating Z α1-antitrypsin lead to emphysema by loss of inhibition of neutrophil elastase. The ideal therapy should prevent polymer formation while preserving inhibitory activity. Here we used mAb technology to identify interactors with Z...

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Identifiers

PMID: 25757566
DOI: 10.1096/fj.14-267351
PMCID: PMC4548814

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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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