Z-α(1)-antitrypsin polymers impose molecular filtration in the endoplasmic reticulum after undergoing phase transition to a solid state

Misfolding of secretory proteins in the endoplasmic reticulum (ER) features in many human diseases. In α(1)-antitrypsin deficiency, the pathogenic Z variant aberrantly assembles into polymers in the hepatocyte ER, leading to cirrhosis. We show that α(1)-antitrypsin polymers undergo a liquid:solid ph...

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Detalles Bibliográficos
Autores principales: Chambers, Joseph E., Zubkov, Nikita, Kubánková, Markéta, Nixon-Abell, Jonathon, Mela, Ioanna, Abreu, Susana, Schwiening, Max, Lavarda, Giulia, López-Duarte, Ismael, Dickens, Jennifer A., Torres, Tomás, Kaminski, Clemens F., Holt, Liam J., Avezov, Edward, Huntington, James A., George-Hyslop, Peter St, Kuimova, Marina K., Marciniak, Stefan J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association for the Advancement of Science 2022
Materias:
Biomedicine and Life Sciences
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8993113/
https://www.ncbi.nlm.nih.gov/pubmed/35394846
http://dx.doi.org/10.1126/sciadv.abm2094

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