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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Dades bibliogràfiques
Publicat a:FASEB J
Autors principals: 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.
Format: Artigo
Idioma:Inglês
Publicat: Federation of American Societies for Experimental Biology 2015
Matèries:
Research Communication
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4548814/
https://ncbi.nlm.nih.gov/pubmed/25757566
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.14-267351
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