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Unveiling the degradative route of the V247M α-sarcoglycan mutant responsible for LGMD-2D
Many membrane and secretory proteins that fail to pass quality control in the endoplasmic reticulum (ER) are dislocated into the cytosol and degraded by the proteasome. In applying rigid rules, however, quality control sometimes discharges proteins that, even though defective, retain their function....
Autores principales: | Bianchini, Elisa, Fanin, Marina, Mamchaoui, Kamel, Betto, Romeo, Sandonà, Dorianna |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4065151/ https://www.ncbi.nlm.nih.gov/pubmed/24565866 http://dx.doi.org/10.1093/hmg/ddu088 |
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