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From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

Molecular biology of the cell, 2016-02, Vol.27 (3), p.424-433

2016

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Cellular mechanisms of membrane protein folding

Nature structural & molecular biology, 2009-06, Vol.16 (16), p.606-612

Skach, William R

2009

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Cotranslational Stabilization of Sec62/63 within the ER Sec61 Translocon Is Controlled by Distinct Substrate-Driven Translocation Events

Molecular cell, 2015-04, Vol.58 (2), p.269-283

2015

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Protein folding and quality control in the endoplasmic reticulum: Recent lessons from yeast and mammalian cell systems

Current opinion in cell biology, 2011-08, Vol.23 (4), p.464-475

2011

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CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates

Nature communications, 2020-08, Vol.11 (1), p.4258-11, Article 4258

2020

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Translational tuning optimizes nascent protein folding in cells

Science (American Association for the Advancement of Science), 2015-04, Vol.348 (6233), p.444-448

2015

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Stepwise Insertion and Inversion of a Type II Signal Anchor Sequence in the Ribosome-Sec61 Translocon Complex

Cell, 2011-07, Vol.146 (1), p.134-147

2011

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Engineered transfer RNAs for suppression of premature termination codons

Nature communications, 2019-02, Vol.10 (1), p.822-822, Article 822

2019

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Endoplasmic Reticulum Protein Quality Control Is Determined by Cooperative Interactions between Hsp/c70 Protein and the CHIP E3 Ligase

The Journal of biological chemistry, 2013-10, Vol.288 (43), p.31069-31079

2013

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Ligand-Driven Vectorial Folding of Ribosome-Bound Human CFTR NBD1

Molecular cell, 2011-03, Vol.41 (6), p.682-692

2011

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A small molecule high throughput screening platform to profile conformational properties of nascent, ribosome-bound proteins

Scientific reports, 2022-02, Vol.12 (1), p.2509-2509, Article 2509

2022

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Mechanisms of CFTR Folding at the Endoplasmic Reticulum

Frontiers in pharmacology, 2012-01, Vol.3, p.201-201

2012

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Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

Nature chemical biology, 2010-01, Vol.6 (1), p.25-33

2010

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expanding role of the ER translocon in membrane protein folding

The Journal of cell biology, 2007-12, Vol.179 (7), p.1333-1335

Skach, William R

2007

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Kinetic Analysis of Ribosome-bound Fluorescent Proteins Reveals an Early, Stable, Cotranslational Folding Intermediate

The Journal of biological chemistry, 2012-01, Vol.287 (4), p.2568-2578

2012

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In vitro modulator responsiveness of 655 CFTR variants found in people with cystic fibrosis

Journal of cystic fibrosis, 2024-07, Vol.23 (4), p.664-675

2024

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A role for the ribosome-associated complex in activation of the IRE1 branch of UPR

Cell reports (Cambridge), 2021-07, Vol.36 (2), p.109366-109366, Article 109366

2021

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Role of Hsc70 binding cycle in CFTR folding and endoplasmic reticulum-associated degradation

Molecular biology of the cell, 2011-08, Vol.22 (16), p.2797-2809

2011

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Cotranslational folding inhibits translocation from within the ribosome-Sec61 translocon complex

Nature structural & molecular biology, 2014-03, Vol.21 (3), p.228-235

2014

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Thermal Instability of ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channel Function: Protection by Single Suppressor Mutations and Inhibiting Channel Activity

Biochemistry (Easton), 2012-06, Vol.51 (25), p.5113-5124

2012

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Sequential triage of transmembrane segments by Sec61α during biogenesis of a native multispanning membrane protein

Nature structural & molecular biology, 2005-10, Vol.12 (10), p.870-878

2005

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Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR

Journal of cystic fibrosis, 2020-03, Vol.19 (Suppl 1), p.S25-S32

2020

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Real-Time Fluorescence Detection of ERAD Substrate Retrotranslocation in a Mammalian In Vitro System

Cell, 2007-06, Vol.129 (5), p.943-955

2007

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Patient-Centered Research Priorities for Pulmonary Nontuberculous Mycobacteria (NTM) Infection. An NTM Research Consortium Workshop Report

Annals of the American Thoracic Society, 2016-09, Vol.13 (9), p.S379-S384

2016

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Aquaporin-4 dynamics in orthogonal arrays in live cells visualized by quantum dot single particle tracking

Molecular biology of the cell, 2008-08, Vol.19 (8), p.3369-3378

2008

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From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

Cellular mechanisms of membrane protein folding

Cotranslational Stabilization of Sec62/63 within the ER Sec61 Translocon Is Controlled by Distinct Substrate-Driven Translocation Events

Protein folding and quality control in the endoplasmic reticulum: Recent lessons from yeast and mammalian cell systems

CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates

Translational tuning optimizes nascent protein folding in cells

Stepwise Insertion and Inversion of a Type II Signal Anchor Sequence in the Ribosome-Sec61 Translocon Complex

Engineered transfer RNAs for suppression of premature termination codons

Endoplasmic Reticulum Protein Quality Control Is Determined by Cooperative Interactions between Hsp/c70 Protein and the CHIP E3 Ligase

Ligand-Driven Vectorial Folding of Ribosome-Bound Human CFTR NBD1

A small molecule high throughput screening platform to profile conformational properties of nascent, ribosome-bound proteins

Mechanisms of CFTR Folding at the Endoplasmic Reticulum

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

expanding role of the ER translocon in membrane protein folding

Kinetic Analysis of Ribosome-bound Fluorescent Proteins Reveals an Early, Stable, Cotranslational Folding Intermediate

In vitro modulator responsiveness of 655 CFTR variants found in people with cystic fibrosis

A role for the ribosome-associated complex in activation of the IRE1 branch of UPR

Role of Hsc70 binding cycle in CFTR folding and endoplasmic reticulum-associated degradation

Cotranslational folding inhibits translocation from within the ribosome-Sec61 translocon complex

Thermal Instability of ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channel Function: Protection by Single Suppressor Mutations and Inhibiting Channel Activity

Sequential triage of transmembrane segments by Sec61α during biogenesis of a native multispanning membrane protein

Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR

Real-Time Fluorescence Detection of ERAD Substrate Retrotranslocation in a Mammalian In Vitro System

Patient-Centered Research Priorities for Pulmonary Nontuberculous Mycobacteria (NTM) Infection. An NTM Research Consortium Workshop Report

Aquaporin-4 dynamics in orthogonal arrays in live cells visualized by quantum dot single particle tracking

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