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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 f...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

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

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-25757566-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

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