Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect

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

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Accurate prediction of cellular co-translational folding indicates proteins can switch from post- to co-translational folding

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∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis

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Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

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Translational tuning optimizes nascent protein folding in cells

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Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis

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Understanding the Influence of Codon Translation Rates on Cotranslational Protein Folding

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Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation

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VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR after Its Partial Rescue to the Cell Surface

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Quantitative Proteomic Profiling Reveals Differentially Regulated Proteins in Cystic Fibrosis Cells

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Optimizing membrane-protein biogenesis through nonoptimal-codon usage

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Local slowdown of translation by nonoptimal codons promotes nascent-chain recognition by SRP in vivo

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Molecular insights into the interaction of the ribosomal stalk protein with elongation factor 1α

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Phosphorylation of Initiation Factor eIF2 in Response to Stress Conditions Is Mediated by Acidic Ribosomal P1/P2 Proteins in Saccharomyces cerevisiae

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Some gating potentiators, including VX-770, diminish  F508-CFTR functional expression

(2014) G. Veit et al.   Science Translational Medicine

Potentiator ivacaftor abrogates pharmacological correction of  F508 CFTR in cystic fibrosis

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Modulation of the Maladaptive Stress Response to Manage Diseases of Protein Folding

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Efficient delivery of RNA interference oligonucleotides to polarized airway epithelia in vitro

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Corrector VX-809 stabilizes the first transmembrane domain of CFTR

(2013) Tip W. Loo et al.   BIOCHEMICAL PHARMACOLOGY

Ubiquitination-dependent quality control of hERG K+ channel with acquired and inherited conformational defect at the plasma membrane

(2013) Pirjo M. Apaja et al.   MOLECULAR BIOLOGY OF THE CELL

VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1

(2013) Hong Yu Ren et al.   MOLECULAR BIOLOGY OF THE CELL

Non-optimal codon usage affects expression, structure and function of clock protein FRQ

(2013) Mian Zhou et al.   NATURE

Structures of the human and Drosophila 80S ribosome

(2013) Andreas M. Anger et al.   NATURE

Mechanism-based corrector combination restores ΔF508-CFTR folding and function

(2013) Tsukasa Okiyoneda et al.   Nature Chemical Biology

Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene

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Carboxy terminal modifications of the P0 protein reveal alternative mechanisms of nuclear ribosomal stalk assembly

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Method for Quantitative Study of Airway Functional Microanatomy Using Micro-Optical Coherence Tomography

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Yeast Reveal a "Druggable" Rsp5/Nedd4 Network that Ameliorates  -Synuclein Toxicity in Neurons

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Novel pharmacological strategies to treat cystic fibrosis

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Correction of Both NBD1 Energetics and Domain Interface Is Required to Restore ΔF508 CFTR Folding and Function

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Ribosome Deficiency Protects Against ER Stress in Saccharomyces cerevisiae

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A Novel Approach to Recovery of Function of Mutant Proteins by Slowing Down Translation

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Silent Substitutions Predictably Alter Translation Elongation Rates and Protein Folding Efficiencies

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Proinflammatory cytokine secretion is suppressed by TMEM16A or CFTR channel activity in human cystic fibrosis bronchial epithelia

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A yeast phenomic model for the gene interaction network modulating CFTR-ΔF508 protein biogenesis

(2012) Raymond J Louie et al.   Genome Medicine

ROCK Inhibitor and Feeder Cells Induce the Conditional Reprogramming of Epithelial Cells

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Ribosome Profiling of Mouse Embryonic Stem Cells Reveals the Complexity and Dynamics of Mammalian Proteomes

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V2 Vasopressin Receptor (V2R) Mutations in Partial Nephrogenic Diabetes Insipidus Highlight Protean Agonism of V2R Antagonists

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New Scenarios of Protein Folding Can Occur on the Ribosome

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

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The Ribosome Modulates Nascent Protein Folding

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Functional Links Between A  Toxicity, Endocytic Trafficking, and Alzheimer's Disease Risk Factors in Yeast

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Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator

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Intragenic Suppressing Mutations Correct the Folding and Intracellular Traffic of Misfolded Mutants of Yor1p, a Eukaryotic Drug Transporter

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Quality control for unfolded proteins at the plasma membrane

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Inhibition of eukaryotic translation elongation by cycloheximide and lactimidomycin

(2010) Tilman Schneider-Poetsch et al.   Nature Chemical Biology

Modelling neurodegeneration in Saccharomyces cerevisiae: why cook with baker's yeast?

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ΔF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers

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Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

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Emergent Properties of Proteostasis in Managing Cystic Fibrosis

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N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic

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

(2009) Darren M Hutt et al.   Nature Chemical Biology

The ribosome as a platform for co-translational processing, folding and targeting of newly synthesized proteins

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Tertiary interactions within the ribosomal exit tunnel

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

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Stringent Mating-Type-Regulated Auxotrophy Increases the Accuracy of Systematic Genetic Interaction Screens with Saccharomyces cerevisiae Mutant Arrays

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Molecular pathogenesis of megalencephalic leukoencephalopathy with subcortical cysts: mutations in MLC1 cause folding defects

(2008) A. Duarri et al.   HUMAN MOLECULAR GENETICS

Structural and functional characterization of the amino terminal domain of the yeast ribosomal stalk P1 and P2 proteins

(2008) Verónica Briceño et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Synonymous Mutations and Ribosome Stalling Can Lead to Altered Folding Pathways and Distinct Minima

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Bioinformatics enrichment tools: paths toward the comprehensive functional analysis of large gene lists

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