Chaperone-Independent Peripheral Quality Control of CFTR by RFFL E3 Ligase (original) (raw)

Rescue of F508del-CFTR by RXR motif inactivation triggers proteome modulation associated with the unfolded protein response

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2019

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Kai Du

The Journal of cell biology, 2004

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Regulated recycling of mutant CFTR is partially restored by pharmacological treatment

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Activation of the Unfolded Protein Response by F508 CFTR

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Most F508del-CFTR Is Targeted to Degradation at an Early Folding Checkpoint and Independently of Calnexin

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Molecular and Cellular Biology, 2005

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Chemical Rescue of F508-CFTR Mimics Genetic Repair in Cystic Fibrosis Bronchial Epithelial Cells

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Molecular & Cellular Proteomics, 2008

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Gp78 Cooperates with RMA1 in Endoplasmic Reticulum-associated Degradation of CFTRΔF508

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Molecular Biology of the Cell, 2008

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Methods in molecular biology (Clifton, N.J.), 2011

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CFTR Expression Regulation by the Unfolded Protein Response

Rafal Bartoszewski

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Interference with ubiquitination in CFTR modifies stability of core glycosylated and cell surface pools

Jordan Despanie

Molecular and Cellular Biology, 2014

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Valeria Villella

Molecular and Cellular Pediatrics, 2016

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High-content Functional Screen to Identify Proteins that Correct F508del-CFTR Function

Diane Ly

Molecular & Cellular Proteomics, 2008

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Roscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanism

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British Journal of Pharmacology, 2014

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Hsp90 Cochaperone Aha1 Downregulation Rescues Misfolding of CFTR in Cystic Fibrosis

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Folding Status Is Determinant over Traffic-Competence in Defining CFTR Interactors in the Endoplasmic Reticulum

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Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis

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Cellular Physiology and Biochemistry, 2017

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Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition

Brian Spencer, Eliezer Masliah, Stefano Guido, Speranza Esposito, Manuela Gavina

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Pharmacological Correctors of Mutant CFTR Mistrafficking

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p97 functions as an auxiliary factor to facilitate TM domain extraction during CFTR ER-associated degradation

David Pitonzo

The EMBO Journal, 2006

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Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment

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Endocytic trafficking of CFTR in health and disease

Mark Silvis

Journal of Cystic Fibrosis, 2007

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Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites

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Rare Trafficking CFTR Mutations Involve Distinct Cellular Retention Machineries and Require Different Rescuing Strategies

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Functional Rescue of F508del-CFTR Using Small Molecule Correctors

Saumel Ahmadi

Frontiers in Pharmacology, 2012

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CFTR mutations altering CFTR fragmentation

Simão Luz

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