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Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases

Many germ line diseases stem from a relatively minor disturbance in mutant protein endoplasmic reticulum (ER) 3D assembly. Chaperones are recruited which, on failure to correct folding, sort the mutant for retrotranslocation and cytosolic proteasomal degradation (ER-associated degradation-ERAD), to...

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Detalles Bibliográficos
Autores principales: Adnan, Humaira, Zhang, Zhenbo, Park, Hyun-Joo, Tailor, Chetankumar, Che, Clare, Kamani, Mustafa, Spitalny, George, Binnington, Beth, Lingwood, Clifford
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5147855/
https://www.ncbi.nlm.nih.gov/pubmed/27935997
http://dx.doi.org/10.1371/journal.pone.0166948