A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnover

The accumulation of serpin oligomers and polymers within the endoplasmic reticulum (ER) causes cellular injury in patients with the classical form α1-antitrypsin deficiency (ATD). To better understand the cellular and molecular genetic aspects of this disorder, we generated transgenic C. elegans str...

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Bibliografische gegevens
Hoofdauteurs: Long, Olivia S., Benson, Joshua A., Kwak, Joon Hyeok, Luke, Cliff J., Gosai, Sager J., O'Reilly, Linda P., Wang, Yan, Li, Jie, Vetica, Anne C., Miedel, Mark T., Stolz, Donna B., Watkins, Simon C., Züchner, Stephan, Perlmutter, David H., Silverman, Gary A., Pak, Stephen C.
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: Oxford University Press 2014
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Online toegang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4159155/
https://ncbi.nlm.nih.gov/pubmed/24838286
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddu235
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