The molecular species responsible for α(1)‐antitrypsin deficiency are suppressed by a small molecule chaperone

The formation of ordered Z (Glu342Lys) α(1)‐antitrypsin polymers in hepatocytes is central to liver disease in α(1)‐antitrypsin deficiency. In vitro experiments have identified an intermediate conformational state (M*) that precedes polymer formation, but this has yet to be identified in vivo. Moreo...

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Detalles Bibliográficos
Autores principales: Ronzoni, Riccardo, Heyer‐Chauhan, Nina, Fra, Annamaria, Pearce, Andrew C., Rüdiger, Martin, Miranda, Elena, Irving, James A., Lomas, David A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Original Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8436759/
https://www.ncbi.nlm.nih.gov/pubmed/33058391
http://dx.doi.org/10.1111/febs.15597

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8436759/
https://www.ncbi.nlm.nih.gov/pubmed/33058391
http://dx.doi.org/10.1111/febs.15597

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