Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator
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Summary
The results unravel an unexpected link between CFTR protein and function, the latter regulating the levels of CFTR surface expression in a positive feed-forward loop, and highlight CFTR as a pivot of proteostasis in bronchial epithelial cells.
- Type
- article
- Published
- 2013-05-17
- Cited by
- 56
- References
- 65
- Access
- Open access
- OpenAlex
- https://openalex.org/W1968018959
- Semantic Scholar
- https://api.semanticscholar.org/CorpusID:23695183
Keywords
Proteostasis, Cystic fibrosis transmembrane conductance regulator, Cell biology, Cystic fibrosis, Ivacaftor
References
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Cited by
- Autophagy and cellular immune responses.
- Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation
- Chloride transporters and receptor‐mediated endocytosis in the renal proximal tubule
- Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation
- Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis
- A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR
- Studies on Respiratory Disorders
- CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis
- Metabolic interactions between cysteamine and epigallocatechin gallate
- Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation
- High-expressing cystic fibrosis transmembrane conductance regulator interacts with histone deacetylase 2 to promote the development of Ph+ leukemia through the HDAC2-mediated PTEN pathway.
- Thymosin α1 represents a potential potent single molecule-based therapy for cystic fibrosis
- Bile acids regulate colonic epithelial barrier function: implications for pathogenesis and therapy of inflammatory bowel disease
- Strategies for the etiological therapy of cystic fibrosis
- Repurposing therapies for the personalised treatment of cystic fibrosis
- Transglutaminase type 2 in the regulation of proteostasis
- Cellular proteostasis: a new twist in the action of thymosin α1
- A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease
- FKBP8 Enhances Protein Stability of the CLC-1 Chloride Channel at the Plasma Membrane
- Therapeutic modulation of autophagy: which disease comes first?
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