Conformational and Temperature-sensitive Stability Defects of the ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator in Post-endoplasmic Reticulum Compartments
Open Access
- 1 March 2001
- journal article
- Published by Elsevier
- Vol. 276 (12) , 8942-8950
- https://doi.org/10.1074/jbc.m009172200
Abstract
No abstract availableKeywords
This publication has 41 references indexed in Scilit:
- Expression of Delta F508 Cystic Fibrosis Transmembrane Conductance Regulator Protein and Related Chloride Transport Properties in the Gallbladder Epithelium From Cystic Fibrosis PatientsHepatology, 1999
- Aggregation As a Determinant of Protein Fate in Post-Golgi Compartments: Role of the Luminal Domain of Furin in Lysosomal TargetingThe Journal of cell biology, 1997
- A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.Journal of Clinical Investigation, 1996
- Alteration of the Cystic Fibrosis Transmembrane Conductance Regulator Folding PathwayJournal of Biological Chemistry, 1996
- Glycerol Reverses the Misfolding Phenotype of the Most Common Cystic Fibrosis MutationJournal of Biological Chemistry, 1996
- Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutationNature, 1991
- Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus OocytesScience, 1991
- Identification of the Cystic Fibrosis Gene: Chromosome Walking and JumpingScience, 1989
- Identification of the Cystic Fibrosis Gene: Genetic AnalysisScience, 1989
- Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNAScience, 1989