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A single-chain variable fragment intrabody prevents intracellular polymerization of Z α(1)-antitrypsin while allowing its antiproteinase activity

Mutant Z α(1)-antitrypsin (E342K) accumulates as polymers within the endoplasmic reticulum (ER) of hepatocytes predisposing to liver disease, whereas low levels of circulating Z α(1)-antitrypsin lead to emphysema by loss of inhibition of neutrophil elastase. The ideal therapy should prevent polymer...

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Detalles Bibliográficos
Autores principales:	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.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Federation of American Societies for Experimental Biology 2015
Materias:	Research Communication
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4548814/ https://www.ncbi.nlm.nih.gov/pubmed/25757566 http://dx.doi.org/10.1096/fj.14-267351

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