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...
Main Authors: | , , , , , , , , |
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Format: | Online |
Language: | English |
Published: |
Public Library of Science
2016
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Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5147855/ |