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XBP1 signalling is essential for alleviating mutant protein aggregation in ER-stress related skeletal disease
The unfolded protein response (UPR) is a conserved cellular response to the accumulation of proteinaceous material in endoplasmic reticulum (ER), active both in health and disease to alleviate cellular stress and improve protein folding. Multiple epiphyseal dysplasia (EDM5) is a genetic skeletal con...
Autores principales: | Piróg, Katarzyna A., Dennis, Ella P., Hartley, Claire L., Jackson, Robert M., Soul, Jamie, Schwartz, Jean-Marc, Bateman, John F., Boot-Handford, Raymond P., Briggs, Michael D. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625722/ https://www.ncbi.nlm.nih.gov/pubmed/31260448 http://dx.doi.org/10.1371/journal.pgen.1008215 |
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