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Hepatocyte proteomes reveal the role of protein disulfide isomerase 4 in alpha 1-antitrypsin deficiency
BACKGROUND & AIMS: A single point mutation in the Z-variant of alpha 1-antitrypsin (Z-AAT) alone can lead to both a protein folding and trafficking defect, preventing its exit from the endoplasmic reticulum (ER), and the formation of aggregates that are retained as inclusions within the ER of he...
Autores principales: | Karatas, Esra, Raymond, Anne-Aurélie, Leon, Céline, Dupuy, Jean-William, Di-Tommaso, Sylvaine, Senant, Nathalie, Collardeau-Frachon, Sophie, Ruiz, Mathias, Lachaux, Alain, Saltel, Frédéric, Bouchecareilh, Marion |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8192868/ https://www.ncbi.nlm.nih.gov/pubmed/34151245 http://dx.doi.org/10.1016/j.jhepr.2021.100297 |
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