Article
Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.
PloS one - 1 Jan 2016
Adnan Humaira, Zhang Zhenbo, Park Hyun-Joo, Tailor Chetankumar, Che Clare, Kamani Mustafa, Spitalny George, Binnington Beth, Lingwood Clifford
Abstract excerpt
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 initiate/exacerbate deficiency-disease symptoms. Several bacterial (and plant) subunit toxins, retrograde transport...
Topics
- Blotting, Western
- Cholera Toxin
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- Endoplasmic Reticulum
- Endoplasmic Reticulum-Associated Degradation
- HEK293 Cells
- Humans
- Microscopy, Fluorescence
- Models, Biological
- Mutation
- Protein Folding