| scholarly article | Q13442814 |
| P50 | author | Eric Sorscher | Q5387551 |
| Jeffery W. Kelly | Q6175781 | ||
| Joel M. Gottesfeld | Q42410767 | ||
| Sabrina Noel | Q56600287 | ||
| Ana R Grant | Q57326027 | ||
| Tsukasa Okiyoneda | Q59661095 | ||
| Raymond A Frizzell | Q87032262 | ||
| Martina Gentzsch | Q89449607 | ||
| William R Skach | Q98733437 | ||
| Gerard Manning | Q28958324 | ||
| Gergely L Lukacs | Q38322272 | ||
| P2093 | author name string | John R Yates | |
| David Herman | |||
| Joseph M Pilewski | |||
| William E Balch | |||
| Philip J Thomas | |||
| Andre Schmidt | |||
| John R Riordan | |||
| Jeanne Matteson | |||
| Yoshihiro Matsumura | |||
| Darren M Hutt | |||
| Wendy Kellner | |||
| Ben Hoch | |||
| P2860 | cites work | HDAC6 regulates Hsp90 acetylation and chaperone-dependent activation of glucocorticoid receptor | Q24303612 |
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| Adapting proteostasis for disease intervention | Q28131818 | ||
| Human HDAC7 histone deacetylase activity is associated with HDAC3 in vivo | Q28207885 | ||
| Evidence for direct CFTR inhibition by CFTR(inh)-172 based on Arg347 mutagenesis. | Q39998495 | ||
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| Pharmacoproteomics of 4-phenylbutyrate-treated IB3-1 cystic fibrosis bronchial epithelial cells. | Q40309578 | ||
| Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers | Q40364800 | ||
| Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathway | Q40756369 | ||
| Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP | Q40794305 | ||
| Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity. | Q45300254 | ||
| Episomal expression of wild-type CFTR corrects cAMP-dependent chloride transport in respiratory epithelial cells | Q45881865 | ||
| Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosis. | Q53608593 | ||
| New insights into cystic fibrosis: molecular switches that regulate CFTR | Q56680230 | ||
| An Adaptable Standard for Protein Export from the Endoplasmic Reticulum | Q57445077 | ||
| Identification of Two New Synthetic Histone Deacetylase Inhibitors That Modulate Globin Gene Expression in Erythroid Cells from Healthy Donors and Patients with Thalassemia | Q58862254 | ||
| Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding | Q73427493 | ||
| Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia | Q74061164 | ||
| A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function | Q74225407 | ||
| Histone deacetylase inhibitors selectively suppress expression of HDAC7 | Q81311085 | ||
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| Histone deacetylase 7 maintains vascular integrity by repressing matrix metalloproteinase 10 | Q28509194 | ||
| Chemical chaperones reduce ER stress and restore glucose homeostasis in a mouse model of type 2 diabetes | Q29615503 | ||
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| Pathway mapping tools for analysis of high content data | Q31061740 | ||
| Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator | Q34359205 | ||
| Histone deacetylase inhibitors: discovery and development as anticancer agents | Q34470150 | ||
| Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants | Q34563643 | ||
| An acetylation site in the middle domain of Hsp90 regulates chaperone function | Q35729439 | ||
| Histone deacetylase 7 functions as a key regulator of genes involved in both positive and negative selection of thymocytes | Q35949614 | ||
| Anti-rheumatic activities of histone deacetylase (HDAC) inhibitors in vivo in collagen-induced arthritis in rodents. | Q36052572 | ||
| Processing of CFTR: traversing the cellular maze--how much CFTR needs to go through to avoid cystic fibrosis? | Q36070221 | ||
| Unraveling the hidden catalytic activity of vertebrate class IIa histone deacetylases | Q36141408 | ||
| Stress-inducible regulation of heat shock factor 1 by the deacetylase SIRT1 | Q36194366 | ||
| COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code | Q36322645 | ||
| Acute ENaC stimulation by cAMP in a kidney cell line is mediated by exocytic insertion from a recycling channel pool | Q36412575 | ||
| Protein misfolding disorders: pathogenesis and intervention | Q36501365 | ||
| Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis | Q36663366 | ||
| Evidence for airway surface dehydration as the initiating event in CF airway disease | Q36707525 | ||
| The therapeutic uses of chromatin-modifying agents | Q36822457 | ||
| Epigenetic regulation of airway inflammation. | Q36919513 | ||
| A therapeutic role for sirtuins in diseases of aging? | Q36991029 | ||
| The Sirtuin family: therapeutic targets to treat diseases of aging | Q37087540 | ||
| CFTR function and prospects for therapy | Q37096095 | ||
| Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking | Q37196094 | ||
| In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. | Q37374706 | ||
| The proteostasis boundary in misfolding diseases of membrane traffic | Q37587095 | ||
| P433 | issue | 1 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | cell biology | Q7141 |
| cystic fibrosis | Q178194 | ||
| P304 | page(s) | 25-33 | |
| P577 | publication date | 2010-01-01 | |
| P1433 | published in | Nature Chemical Biology | Q904026 |
| P1476 | title | Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis | |
| P478 | volume | 6 |
| Q34305005 | A chaperone trap contributes to the onset of cystic fibrosis |
| Q34305593 | A genomic signature approach to rescue ΔF508-cystic fibrosis transmembrane conductance regulator biosynthesis and function |
| Q35608048 | Acetylation stimulates the epithelial sodium channel by reducing its ubiquitination and degradation |
| Q34323595 | Activation of the stress proteome as a mechanism for small molecule therapeutics |
| Q61803810 | Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease |
| Q36566693 | An innovative approach to the treatment of Gaucher disease and possibly other metabolic disorders of the brain |
| Q34076025 | Applications of proteomic technologies for understanding the premature proteolysis of CFTR. |
| Q91936980 | Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity |
| Q40675816 | Biochemical and biophysical approaches to probe CFTR structure. |
| Q38533943 | Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors |
| Q28073996 | CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis |
| Q90175980 | CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine |
| Q38972431 | CFTR pharmacology. |
| Q36814102 | CFTR: folding, misfolding and correcting the ΔF508 conformational defect. |
| Q27004511 | Chemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosis |
| Q37819121 | Chemical and/or biological therapeutic strategies to ameliorate protein misfolding diseases |
| Q48009483 | Chemical corrector treatment ameliorates increased seizure susceptibility in a mouse model of familial epilepsy |
| Q51031981 | Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis. |
| Q41893111 | Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines. |
| Q35612252 | Compromised mutant EFEMP1 secretion associated with macular dystrophy remedied by proteostasis network alteration |
| Q63640421 | Correcting the F508del-CFTR variant by modulating eukaryotic translation initiation factor 3-mediated translation initiation |
| Q35558817 | Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. |
| Q41411560 | Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis. |
| Q37744944 | Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation |
| Q28073063 | Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives |
| Q34023921 | Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis |
| Q38118742 | Cystic fibrosis transmembrane regulator correctors and potentiators |
| Q39214537 | Dephosphorylation at a conserved SP motif governs cAMP sensitivity and nuclear localization of class IIa histone deacetylases |
| Q37164477 | Development, clinical utility, and place of ivacaftor in the treatment of cystic fibrosis |
| Q93000176 | Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR |
| Q34975364 | Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain |
| Q35790801 | Drug-set enrichment analysis: a novel tool to investigate drug mode of action |
| Q41396117 | Efficient delivery of RNA interference oligonucleotides to polarized airway epithelia in vitro |
| Q34576386 | Emergent properties of proteostasis in managing cystic fibrosis |
| Q64095087 | Emerging Therapeutic Approaches for Cystic Fibrosis. From Gene Editing to Personalized Medicine |
| Q33888627 | Endoplasmic reticulum-associated degradation of Niemann-Pick C1: evidence for the role of heat shock proteins and identification of lysine residues that accept ubiquitin |
| Q33632575 | Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis |
| Q52882350 | Epigenetic Modulation of Collagen 1A1: Therapeutic Implications in Fibrosis and Endometriosis. |
| Q26824651 | Expanding proteostasis by membrane trafficking networks |
| Q28550025 | Exploration of Novel Inhibitors for Class I Histone Deacetylase Isoforms by QSAR Modeling and Molecular Dynamics Simulation Assays |
| Q90736025 | Extracellular Vesicle-Mediated siRNA Delivery, Protein Delivery, and CFTR Complementation in Well-Differentiated Human Airway Epithelial Cells |
| Q64244254 | Folding and Misfolding of Human Membrane Proteins in Health and Disease: From Single Molecules to Cellular Proteostasis |
| Q36574664 | From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations |
| Q35988949 | Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report |
| Q35693982 | Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation. |
| Q34982862 | Genetic therapies for cystic fibrosis lung disease |
| Q36729796 | Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules |
| Q64084368 | HDAC Inhibitors: Therapeutic Potential in Fibrosis-Associated Human Diseases |
| Q36279553 | Hallmarks of therapeutic management of the cystic fibrosis functional landscape. |
| Q35556841 | Hdac-mediated control of endochondral and intramembranous ossification |
| Q52585959 | High-content screen for modifiers of Niemann-Pick Type C disease in patient cells. |
| Q42024732 | Histone Adduction and Its Functional Impact on Epigenetics |
| Q34475885 | Histone deacetylase inhibition destabilizes the multi-potent state of uncommitted adipose-derived mesenchymal stromal cells |
| Q37213816 | Histone deacetylase inhibition promotes osteoblast maturation by altering the histone H4 epigenome and reduces Akt phosphorylation. |
| Q24619820 | Histone deacetylase inhibitor (HDACi) suberoylanilide hydroxamic acid (SAHA)-mediated correction of α1-antitrypsin deficiency |
| Q38855904 | Histone deacetylase inhibitor restores surfactant protein-C expression in alveolar-epithelial type II cells and attenuates bleomycin-induced pulmonary fibrosis in vivo. |
| Q36545633 | Histone deacetylase inhibitors increase glucocerebrosidase activity in Gaucher disease by modulation of molecular chaperones |
| Q34179757 | Histone deacetylase inhibitors influence chemotherapy transport by modulating expression and trafficking of a common polymorphic variant of the ABCG2 efflux transporter |
| Q35641509 | Histone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher disease |
| Q38908331 | Histone deacetylases function as a novel potential therapeutic target for cancer |
| Q34612849 | Histone deacetylases in skeletal development and bone mass maintenance |
| Q36003713 | Human heat shock protein 105/110 kDa (Hsp105/110) regulates biogenesis and quality control of misfolded cystic fibrosis transmembrane conductance regulator at multiple levels |
| Q37768771 | Hydroxamic acids (therapeutics and mechanism): chemistry, acyl nitroso, nitroxyl, reactive oxygen species, and cell signaling |
| Q33738057 | Identification of Synergistic Combinations of F508del Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators |
| Q33807836 | Improved Growth Patterns in Cystic Fibrosis Mice after Loss of Histone Deacetylase 6. |
| Q28554668 | Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics |
| Q47231903 | Inhibition of histone-deacetylase activity rescues inflammatory cystic fibrosis lung disease by modulating innate and adaptive immune responses |
| Q38200933 | Innovative strategies to treat protein misfolding in inborn errors of metabolism: pharmacological chaperones and proteostasis regulators. |
| Q41136041 | Introduction to section IV: biophysical methods to approach CFTR structure. |
| Q34534598 | Long Non-coding RNA BGas Regulates the Cystic Fibrosis Transmembrane Conductance Regulator |
| Q38113749 | Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors |
| Q26765794 | Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis |
| Q35219853 | Measurement of the airway surface liquid volume with simple light refraction microscopy |
| Q47160647 | Measuring the Effect of Histone Deacetylase Inhibitors (HDACi) on the Secretion and Activity of Alpha-1 Antitrypsin. |
| Q37860156 | Mis-trafficking of bicarbonate transporters: implications to human diseases |
| Q37815987 | Modeling general proteostasis: proteome balance in health and disease |
| Q27313626 | Modulation of the maladaptive stress response to manage diseases of protein folding |
| Q37880729 | Molecular chaperones as therapeutic targets to counteract proteostasis defects. |
| Q26826845 | Molecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosis |
| Q83810259 | Mutation-specific cystic fibrosis treatments on verge of approval |
| Q35781735 | Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium |
| Q34269429 | Neurology of inherited glycosylation disorders |
| Q48379148 | Normalization of Hepatic Homeostasis in the Npc1nmf164 Mouse Model of Niemann-Pick Type C Disease Treated with the Histone Deacetylase Inhibitor Vorinostat |
| Q34132264 | Obesity-linked variants of melanocortin-4 receptor are misfolded in the endoplasmic reticulum and can be rescued to the cell surface by a chemical chaperone |
| Q36296274 | Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells |
| Q57490973 | Peripheral Protein Quality Control as a Novel Drug Target for CFTR Stabilizer |
| Q34444239 | Pharmacological Correctors of Mutant CFTR Mistrafficking |
| Q37887169 | Pharmacological therapy for cystic fibrosis: from bench to bedside |
| Q35827545 | Phosphorylation-dependent 14-3-3 protein interactions regulate CFTR biogenesis |
| Q35275697 | Potential Agents for Treating Cystic Fibrosis: Cyclic Tetrapeptides that Restore Trafficking and Activity of ΔF508-CFTR |
| Q40725277 | Progress in therapies for cystic fibrosis |
| Q28743986 | Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model |
| Q27003283 | Protein homeostasis as a therapeutic target for diseases of protein conformation |
| Q37804635 | Proteostasis strategies for restoring alpha1-antitrypsin deficiency |
| Q42790541 | Proteostasis: a new therapeutic paradigm for pulmonary disease |
| Q91173541 | Quantitating the epigenetic transformation contributing to cholesterol homeostasis using Gaussian process |
| Q46094394 | Quantitative Analysis of the Proteome Response to the Histone Deacetylase Inhibitor (HDACi) Vorinostat in Niemann-Pick Type C1 disease. |
| Q41777167 | Quantitative proteomic profiling reveals differentially regulated proteins in cystic fibrosis cells |
| Q49284664 | Recent Progress in CFTR Interactome Mapping and Its Importance for Cystic Fibrosis |
| Q36481817 | Reduced PDZ interactions of rescued ΔF508CFTR increases its cell surface mobility. |
| Q42585054 | Reduced microtubule acetylation in cystic fibrosis epithelial cells |
| Q37960092 | Regulation of airway mucosal hydration |
| Q33960117 | Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling. |
| Q35852856 | Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation |
| Q49885765 | Restoration of mutant hERG stability by inhibition of HDAC6. |
| Q104268677 | Revealing Functional Insights into ER Proteostasis through Proteomics and Interactomics |
| Q28551966 | Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect |
| Q35160700 | Role of Hsc70 binding cycle in CFTR folding and endoplasmic reticulum-associated degradation |
| Q38897940 | Role of STARD4 and NPC1 in intracellular sterol transport |
| Q37718121 | Role of endosomes and lysosomes in human disease. |
| Q34954019 | Roscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanism |
| Q37412871 | SAHA enhances Proteostasis of epilepsy-associated α1(A322D)β2γ2 GABA(A) receptors. |
| Q46353737 | SYVN1, NEDD8, and FBXO2 Proteins Regulate ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitin-mediated Proteasomal Degradation |
| Q54090838 | Silencing Histone Deacetylase 7 Alleviates Transforming Growth Factor-β1-Induced Profibrotic Responses in Fibroblasts Derived from Peyronie's Plaque. |
| Q89479714 | Silencing of the Hsp70-specific nucleotide-exchange factor BAG3 corrects the F508del-CFTR variant by restoring autophagy |
| Q57147773 | Sodium taurocholate cotransporting polypeptide (SLC10A1) deficiency: conjugated hypercholanemia without a clear clinical phenotype |
| Q42650380 | Strategies for the etiological therapy of cystic fibrosis |
| Q34810920 | Suberoylanilide hydroxamic acid (SAHA; vorinostat) causes bone loss by inhibiting immature osteoblasts. |
| Q42558062 | Synonymous codon usage affects the expression of wild type and F508del CFTR. |
| Q34488617 | Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane |
| Q38631561 | Targeting the PI3K/Akt/mTOR signalling pathway in Cystic Fibrosis |
| Q34082569 | Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator |
| Q38839806 | The CF-modifying gene EHF promotes p.Phe508del-CFTR residual function by altering protein glycosylation and trafficking in epithelial cells |
| Q30394029 | The cystic fibrosis transmembrane conductance regulator (CFTR) and its stability |
| Q27015793 | The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology |
| Q39283136 | The effect of various zinc binding groups on inhibition of histone deacetylases 1-11. |
| Q28542639 | The histone deacetylase inhibitor, Vorinostat, represses hypoxia inducible factor 1 alpha expression through translational inhibition |
| Q40859336 | The holy grail of cystic fibrosis research: pharmacological repair of the F508del-CFTR mutation |
| Q47842086 | Toward inclusive therapy with CFTR modulators: Progress and challenges. |
| Q36568529 | Unravelling druggable signalling networks that control F508del-CFTR proteostasis |
| Q37892766 | Update in cystic fibrosis 2010. |
| Q41912848 | mTORC1 activation in podocytes is a critical step in the development of diabetic nephropathy in mice |
| Q40006173 | ∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis |
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