Article
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein.
Cell stress & chaperones - 1 Jun 1996
Brown C R, Hong-Brown L Q, Biwersi J, Verkman A S, Welch W J
Abstract excerpt
Mutations in the cystic fibrosis transmembrane conductance regulator protein (CFTR) often result in a failure of the protein to be properly processed at the level of the endoplasmic reticulum (ER) and subsequently transported to the plasma membrane. The folding defect associated with the most common CFTR mutation (delta F508) has been shown to be temperature sensitive. Incubation of cells expressing delta F508...
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