Advanced search
Publication Cover
Autophagy Volume 10, 2014 - Issue 11
Submit an article Journal homepage
8,539
Views
125
CrossRef citations to date
0
Altmetric
Translational Research Papers

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

Daniela De StefanoEuropean Institute for Research in Cystic Fibrosis; Division of Genetics and Cell Biology; San Raffaele Scientific Institute; Milan, Italy
,
Valeria R VillellaEuropean Institute for Research in Cystic Fibrosis; Division of Genetics and Cell Biology; San Raffaele Scientific Institute; Milan, Italy
,
Speranza EspositoEuropean Institute for Research in Cystic Fibrosis; Division of Genetics and Cell Biology; San Raffaele Scientific Institute; Milan, Italy
,
Antonella ToscoRegional Cystic Fibrosis Center; Pediatric Unit; Department of Translational Medical Sciences; Federico II University; Naples, Italy
,
Angela SepeRegional Cystic Fibrosis Center; Pediatric Unit; Department of Translational Medical Sciences; Federico II University; Naples, Italy
,
Fabiola De GregorioRegional Cystic Fibrosis Center; Pediatric Unit; Department of Translational Medical Sciences; Federico II University; Naples, Italy
,
Laura SalvadoriRegional Cystic Fibrosis Center; Pediatric Unit; Department of Translational Medical Sciences; Federico II University; Naples, Italy
,
Rosa GrassiaOtorhinolaryngology Unit; Monaldi Hospital; Naples, Italy
,
Carlo A LeoneOtorhinolaryngology Unit; Monaldi Hospital; Naples, Italy
,
Giuseppe De RosaDepartment of Pharmacy; School of Pharmacy; Federico II University; Naples, Italy
,
Maria C MaiuriDepartment of Pharmacy; School of Pharmacy; Federico II University; Naples, Italy;Equipe 11 labellisée Ligue contre le Cancer; INSERM U1138; Centre de Recherche des Cordeliers; Paris, France
,
Massimo Pettoello-MantovaniInstitute of Pediatrics; University of Foggia; Foggia, Italy
,
Stefano GuidoDepartment of Chemical, Materials and Production Engineering; Federico II University; Naples, Italy
,
Anna BossiDepartment of Clinical Sciences and Community Health; Unit of Medical Statistics; University of Milan; Italy
,
Anna ZolinDepartment of Clinical Sciences and Community Health; Unit of Medical Statistics; University of Milan; Italy
,
Andrea VenerandoDepartment of Biomedical Science and CNR Institute of Neurosciences; University of Padova; Padua, Italy
,
Lorenzo A PinnaDepartment of Biomedical Science and CNR Institute of Neurosciences; University of Padova; Padua, Italy
,
Anil MehtaDivision of Cardiovascular and Diabetes Medicine; Ninewells Hospital and Medical School; University of Dundee; Dundee, UK
,
Gianni BonaSCDU of Pediatrics; Department of Health Sciences; University of Piemonte Orientale; Novara, Italy
,
Guido KroemerEquipe 11 labellisée Ligue contre le Cancer; INSERM U1138; Centre de Recherche des Cordeliers; Paris, France;Université Paris Descartes; Paris, France;Metabolomics and Cell Biology Platforms; Institut Gustave Roussy; Villejuif, France;Pôle de Biologie; Hôpital Européen Georges Pompidou; AP-HP; Paris, FranceCorrespondence[email protected] [email protected] [email protected]
,
Luigi MaiuriEuropean Institute for Research in Cystic Fibrosis; Division of Genetics and Cell Biology; San Raffaele Scientific Institute; Milan, Italy;SCDU of Pediatrics; Department of Health Sciences; University of Piemonte Orientale; Novara, ItalyCorrespondence[email protected] [email protected] [email protected]
&
Valeria RaiaRegional Cystic Fibrosis Center; Pediatric Unit; Department of Translational Medical Sciences; Federico II University; Naples, ItalyCorrespondence[email protected] [email protected] [email protected]
 show all
Pages 2053-2074 | Received 28 Apr 2014, Accepted 27 Aug 2014, Published online: 18 Dec 2014

Citations (125)

Keep up to date with the latest research on this topic with citation updates for this article.

Subscribe to citation updates

Read on this site (14)

Aarti Pant, Xiaomin Yao, Aude Lavedrine, Christophe Viret, Jacob Dockterman, Swati Chauhan, Chong-Shan Shi, Ravi Manjithaya, Ken Cadwell, Thomas A. Kufer, John H. Kehrl, Jörn Coers, L. David Sibley, Mathias Faure, Gregory A. Taylor & Santosh Chauhan. (2022) Interactions of Autophagy and the Immune System in Health and Diseases. Autophagy Reports 1:1, pages 438-515.
Read now
Elisabeth G. Foerster, Tapas Mukherjee, Liliane Cabral-Fernandes, Juliana D.B. Rocha, Stephen E. Girardin & Dana J. Philpott. (2022) How autophagy controls the intestinal epithelial barrier. Autophagy 18:1, pages 86-103.
Read now
Jie Wang, Lina Yang, Jialing You, Dada Wen, Bo Yang & Canhua Jiang. (2021) Platelet-Derived Growth Factor Regulates the Biological Behavior of Oral Mucosal Fibroblasts by Inducing Cell Autophagy and Its Mechanism. Journal of Inflammation Research 14, pages 3405-3417.
Read now
John W. Hanrahan, Yukiko Sato, Graeme W. Carlile, Gregor Jansen, Jason C. Young & David Y. Thomas. (2019) Cystic Fibrosis: Proteostatic correctors of CFTR trafficking and alternative therapeutic targets.. Expert Opinion on Therapeutic Targets 23:8, pages 711-724.
Read now
Janine Faraj, Manish Bodas, Garrett Pehote, Doug Swanson, Ajit Sharma & Neeraj Vij. (2019) Novel cystamine-core dendrimer-formulation rescues ΔF508-CFTR and inhibits Pseudomonas aeruginosa infection by augmenting autophagy. Expert Opinion on Drug Delivery 16:2, pages 177-186.
Read now
Lorenzo Galluzzi & Guido Kroemer. (2019) Etiological involvement of CFTR in apparently unrelated human diseases. Molecular & Cellular Oncology 6:1.
Read now
Antonella Tosco, Valeria R. Villella, Alice Castaldo, Guido Kroemer, Luigi Maiuri & Valeria Raia. (2018) Repurposing therapies for the personalised treatment of cystic fibrosis. Expert Opinion on Orphan Drugs 6:6, pages 361-373.
Read now
Sabrina Bimonte, Marco Cascella, Vincenzo Schiavone, Farrokh Mehrabi-Kermani & Arturo Cuomo. (2017) The roles of epigallocatechin-3-gallate in the treatment of neuropathic pain: an update on preclinical in vivo studies and future perspectives. Drug Design, Development and Therapy 11, pages 2737-2742.
Read now
Neeraj Vij. (2017) Nano-based rescue of dysfunctional autophagy in chronic obstructive lung diseases. Expert Opinion on Drug Delivery 14:4, pages 483-489.
Read now
Valentina Izzo, Federico Pietrocola, Valentina Sica, Sylvère Durand, Sylvie Lachkar, David Enot, José Manuel Bravo-San Pedro, Alexis Chery, Speranza Esposito, Valeria Raia, Luigi Maiuri, Maria Chiara Maiuri & Guido Kroemer. (2017) Metabolic interactions between cysteamine and epigallocatechin gallate. Cell Cycle 16:3, pages 271-279.
Read now
Béla Z Schmidt, Jérémy B Haaf, Teresinha Leal & Sabrina Noel. (2016) Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives. Clinical Pharmacology: Advances and Applications 8, pages 127-140.
Read now
Mia F. Tazi, Duaa A. Dakhlallah, Kyle Caution, Madelyn M. Gerber, Sheng-Wei Chang, Hany Khalil, Benjamin T. Kopp, Amr E. Ahmed, Kathrin Krause, Ian Davis, Clay Marsh, Amy E. Lovett-Racke, Larry S. Schlesinger, Estelle Cormet-Boyaka & Amal O. Amer. (2016) Elevated Mirc1/Mir17-92 cluster expression negatively regulates autophagy and CFTR (cystic fibrosis transmembrane conductance regulator) function in CF macrophages. Autophagy 12:11, pages 2026-2037.
Read now
Andrew R. Turnbull & Jane C. Davies. (2016) New drug developments in the management of cystic fibrosis lung disease. Expert Opinion on Pharmacotherapy 17:8, pages 1103-1112.
Read now
Barbara Dhooghe, Jérémy Boris Haaf, Sabrina Noel & Teresinha Leal. (2016) Strategies in early clinical development for the treatment of basic defects of cystic fibrosis. Expert Opinion on Investigational Drugs 25:4, pages 423-436.
Read now

Articles from other publishers (111)

Md Meraj Anjum, Krishna Kumar Patel, Sankha Bhattacharya, Dilip Kumar Arya, Prashant Pandey, Vijayakumar MR, Sanjay Singh & Paruvathanahalli Siddalingam Rajinikanth. (2023) Overcoming barriers in cystic fibrosis therapy through inhalational lipid nanoparticles: Challenges and advances. Journal of Drug Delivery Science and Technology 89, pages 105068.
Crossref
Irene Brusa, Elvira Sondo, Emanuela Pesce, Valeria Tomati, Dario Gioia, Federico Falchi, Beatrice Balboni, Jose Antonio Ortega Martínez, Marina Veronesi, Elisa Romeo, Natasha Margaroli, Maurizio Recanatini, Stefania Girotto, Nicoletta Pedemonte, Marinella Roberti & Andrea Cavalli. (2023) Innovative Strategy toward Mutant CFTR Rescue in Cystic Fibrosis: Design and Synthesis of Thiadiazole Inhibitors of the E3 Ligase RNF5. Journal of Medicinal Chemistry 66:14, pages 9797-9822.
Crossref
Mateusz Kciuk, Manzar Alam, Nemat Ali, Summya Rashid, Pola Głowacka, Rajamanikandan Sundaraj, Ismail Celik, Esam Bashir Yahya, Amit Dubey, Enfale Zerroug & Renata Kontek. (2023) Epigallocatechin-3-Gallate Therapeutic Potential in Cancer: Mechanism of Action and Clinical Implications. Molecules 28:13, pages 5246.
Crossref
Luca Occhigrossi, Federica Rossin, Valeria Rachela Villella, Speranza Esposito, Carlo Abbate, Manuela D’Eletto, Maria Grazia Farrace, Antonella Tosco, Roberta Nardacci, Gian Maria Fimia, Valeria Raia & Mauro Piacentini. (2023) The STING/TBK1/IRF3/IFN type I pathway is defective in cystic fibrosis. Frontiers in Immunology 14.
Crossref
Shaorong Luan, Yongjun Chen, Xiaohua Wang, Dongmei Yan, Jialin Xu, Hairong Cui & Qingchun Huang. (2022) Synergy of cystamine and pyraclostrobin against Fusarium graminearum involves membrane permeability mitigation and autophagy enhancement. Pesticide Biochemistry and Physiology 188, pages 105287.
Crossref
Paola Fossa, Matteo Uggeri, Alessandro Orro, Chiara Urbinati, Alessandro Rondina, Maria Milanesi, Nicoletta Pedemonte, Emanuela Pesce, Rita Padoan, Robert C. Ford, Xin Meng, Marco Rusnati & Pasqualina D’Ursi. (2022) Virtual Drug Repositioning as a Tool to Identify Natural Small Molecules That Synergize with Lumacaftor in F508del-CFTR Binding and Rescuing. International Journal of Molecular Sciences 23:20, pages 12274.
Crossref
Marianna Carinci, Laura Palumbo, Giulia Pellielo, Esther Densu Agyapong, Giampaolo Morciano, Simone Patergnani, Carlotta Giorgi, Paolo Pinton & Alessandro Rimessi. (2022) The Multifaceted Roles of Autophagy in Infectious, Obstructive, and Malignant Airway Diseases. Biomedicines 10:8, pages 1944.
Crossref
Marjolein M. Ensinck & Marianne S. Carlon. (2022) One Size Does Not Fit All: The Past, Present and Future of Cystic Fibrosis Causal Therapies. Cells 11:12, pages 1868.
Crossref
Irene Brusa, Elvira Sondo, Federico Falchi, Nicoletta Pedemonte, Marinella Roberti & Andrea Cavalli. (2022) Proteostasis Regulators in Cystic Fibrosis: Current Development and Future Perspectives. Journal of Medicinal Chemistry 65:7, pages 5212-5243.
Crossref
Giacomo Picci, Silvia Marchesan & Claudia Caltagirone. (2022) Ion Channels and Transporters as Therapeutic Agents: From Biomolecules to Supramolecular Medicinal Chemistry. Biomedicines 10:4, pages 885.
Crossref
Anabela S. Ramalho, Mieke Boon, Marijke Proesmans, François Vermeulen, Marianne S. Carlon & Kris Boeck. (2022) Assays of CFTR Function In Vitro, Ex Vivo and In Vivo. International Journal of Molecular Sciences 23:3, pages 1437.
Crossref
Douglas Fraser-Pitt & Derry Mercer. (2021) What role for cysteamine in the defence against infection?. Emerging Topics in Life Sciences 5:5, pages 629-635.
Crossref
Verónica Roxana Flores-Vega, Silvia Yalid Vargas-Roldán, José Luis Lezana-Fernández, Ricardo Lascurain, José Ignacio Santos-Preciado & Roberto Rosales-Reyes. (2021) Bacterial Subversion of Autophagy in Cystic Fibrosis. Frontiers in Cellular and Infection Microbiology 11.
Crossref
Douglas J. Fraser-Pitt, Stephen K. Dolan, David Toledo-Aparicio, Jessica G. Hunt, Daniel W. Smith, Niamh Lacy-Roberts, Piumi Sara Nupe Hewage, Teodora N. Stoyanova, Erin Manson, Kevin McClean, Neil F. Inglis, Derry K. Mercer & Deborah A. O’Neil. (2021) Cysteamine Inhibits Glycine Utilisation and Disrupts Virulence in Pseudomonas aeruginosa. Frontiers in Cellular and Infection Microbiology 11.
Crossref
Luca Fasolato, Massimiliano Magro, Giorgio Cozza, Ferruccio Sbarra, Simone Molinari, Enrico Novelli, Fabio Vianello & Andrea Venerando. (2021) An Iron Shield to Protect Epigallocatehin-3-Gallate from Degradation: Multifunctional Self-Assembled Iron Oxide Nanocarrier Enhances Protein Kinase CK2 Intracellular Targeting and Inhibition. Pharmaceutics 13:8, pages 1266.
Crossref
Esra Karatas, Anne-Aurélie Raymond, Céline Leon, Jean-William Dupuy, Sylvaine Di-Tommaso, Nathalie Senant, Sophie Collardeau-Frachon, Mathias Ruiz, Alain Lachaux, Frédéric Saltel & Marion Bouchecareilh. (2021) Hepatocyte proteomes reveal the role of protein disulfide isomerase 4 in alpha 1-antitrypsin deficiency. JHEP Reports 3:4, pages 100297.
Crossref
Luca Occhigrossi, Manuela D’Eletto, Nickolai Barlev & Federica Rossin. (2021) The Multifaceted Role of HSF1 in Pathophysiology: Focus on Its Interplay with TG2. International Journal of Molecular Sciences 22:12, pages 6366.
Crossref
Anna Atlante & Daniela Valenti. (2021) A Walk in the Memory, from the First Functional Approach up to Its Regulatory Role of Mitochondrial Bioenergetic Flow in Health and Disease: Focus on the Adenine Nucleotide Translocator. International Journal of Molecular Sciences 22:8, pages 4164.
Crossref
Vincent J. Venditto, Dalia Haydar, Ahmed Abdel-Latif, John C. Gensel, Michael I. Anstead, Michelle G. Pitts, Jarrod Creameans, Timothy J. Kopper, Chi Peng & David J. Feola. (2021) Immunomodulatory Effects of Azithromycin Revisited: Potential Applications to COVID-19. Frontiers in Immunology 12.
Crossref
Neeraj Vij. (2021) Prognosis-Based Early Intervention Strategies to Resolve Exacerbation and Progressive Lung Function Decline in Cystic Fibrosis. Journal of Personalized Medicine 11:2, pages 96.
Crossref
Kavisha Arora, Pramodha Liyanage, Qing Zhong & Anjaparavanda P. Naren. (2020) A SNARE protein Syntaxin 17 captures CFTR to potentiate autophagosomal clearance under stress. The FASEB Journal 35:2.
Crossref
Christian Borgo & Maria Ruzzene. 2021. Protein Kinases in Drug Discovery. Protein Kinases in Drug Discovery 23 46 .
Guadalupe Martínez-Chacón, Francisco Javier Vela, José Luis Campos, Elena Abellán, Sokhna M. S. Yakhine-Diop & Alberto Ballestín. (2020) Autophagy modulation in animal models of corneal diseases: a systematic review. Molecular and Cellular Biochemistry 474:1-2, pages 41-55.
Crossref
Antonio Recchiuti, Elisa Isopi, Mario Romano & Domenico Mattoscio. (2020) Roles of Specialized Pro-Resolving Lipid Mediators in Autophagy and Inflammation. International Journal of Molecular Sciences 21:18, pages 6637.
Crossref
Garrett Pehote & Neeraj Vij. (2020) Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations. Cells 9:9, pages 1952.
Crossref
Giulio Cabrini, Alessandro Rimessi, Monica Borgatti, Ilaria Lampronti, Alessia Finotti, Paolo Pinton & Roberto Gambari. (2020) Role of Cystic Fibrosis Bronchial Epithelium in Neutrophil Chemotaxis. Frontiers in Immunology 11.
Crossref
Matthew D. Strub & Paul B. McCray, Jr.. (2020) Transcriptomic and Proteostasis Networks of CFTR and the Development of Small Molecule Modulators for the Treatment of Cystic Fibrosis Lung Disease. Genes 11:5, pages 546.
Crossref
Claudio D'Amore, Christian Borgo, Valentina Bosello-Travain, Jordi Vilardell, Valentina Salizzato, Lorenzo A. Pinna, Andrea Venerando & Mauro Salvi. (2020) Deciphering the role of protein kinase CK2 in the maturation/stability of F508del-CFTR. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1866:3, pages 165611.
Crossref
Jennifer Danielsson, Aisha S. Kuforiji, Gene T. Yocum, Yi Zhang, Dingbang Xu, George Gallos & Charles W. EmalaSr.Sr.. (2020) Agonism of the TMEM16A calcium-activated chloride channel modulates airway smooth muscle tone. American Journal of Physiology-Lung Cellular and Molecular Physiology 318:2, pages L287-L295.
Crossref
Ivana Palucci, Giuseppe Maulucci, Flavio De Maio, Michela Sali, Alessandra Romagnoli, Linda Petrone, Gian Maria Fimia, Maurizio Sanguinetti, Delia Goletti, Marco De Spirito, Mauro Piacentini & Giovanni Delogu. (2020) Inhibition of Transglutaminase 2 as a Potential Host-Directed Therapy Against Mycobacterium tuberculosis. Frontiers in Immunology 10.
Crossref
Antonella Tosco, Valeria R. Villella, Valeria Raia, Guido Kroemer & Luigi Maiuri. (2020) Cystic Fibrosis: New Insights into Therapeutic Approaches. Current Respiratory Medicine Reviews 15:3, pages 174-186.
Crossref
S. Memmert, A. V. B. Nogueira, A. Damanaki, M. Nokhbehsaim, B. Rath-Deschner, W. Götz, L. Gölz, J. A. Cirelli, A. Till, A. Jäger & J. Deschner. (2019) Regulation of the autophagy-marker Sequestosome 1 in periodontal cells and tissues by biomechanical loadingRegulation des Autophagie-Markers Sequestosom 1 durch biomechanische Kräfte in parodontalen Zellen und Geweben. Journal of Orofacial Orthopedics / Fortschritte der Kieferorthopädie 81:1, pages 10-21.
Crossref
Cecilia Velino, Francesca Carella, Alessio Adamiano, Maurizio Sanguinetti, Alberto Vitali, Daniele Catalucci, Francesca Bugli & Michele Iafisco. (2019) Nanomedicine Approaches for the Pulmonary Treatment of Cystic Fibrosis. Frontiers in Bioengineering and Biotechnology 7.
Crossref
Bindu D. Paul & Solomon H. Snyder. (2019) Therapeutic Applications of Cysteamine and Cystamine in Neurodegenerative and Neuropsychiatric Diseases. Frontiers in Neurology 10.
Crossref
Luigi Maiuri, Valeria Raia, Mauro Piacentini, Antonella Tosco, Valeria Rachela Villella & Guido Kroemer. (2019) Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease . Oncotarget 10:43, pages 4492-4500.
Crossref
Valeria R. Villella, Antonella Tosco, Speranza Esposito, Eleonora Ferrari, Gianni Bona, Guido Kroemer, Valeria Raia & Luigi Maiuri. (2019) Personalization of therapies in rare diseases: a translational approach for the treatment of cystic fibrosis. Minerva Pediatrica 71:4.
Crossref
Kyle Caution, Alexander Pan, Kathrin Krause, Asmaa Badr, Kaitlin Hamilton, Anup Vaidya, Hawin Gosu, Kylene Daily, Shady Estfanous, Mikhail A. Gavrilin, Mark E. Drew, Estelle Cormet-Boyaka, Xi Chen, David E. Frankhouser, Ralf Bundschuh, Pearlly Yan, Duaa Dakhlallah & Amal O. Amer. (2019) Methylomic correlates of autophagy activity in cystic fibrosis. Journal of Cystic Fibrosis 18:4, pages 491-500.
Crossref
Justin T. Marinko, Hui Huang, Wesley D. Penn, John A. Capra, Jonathan P. Schlebach & Charles R. Sanders. (2019) Folding and Misfolding of Human Membrane Proteins in Health and Disease: From Single Molecules to Cellular Proteostasis. Chemical Reviews 119:9, pages 5537-5606.
Crossref
Speranza Esposito, Valeria Rachela Villella, Eleonora Ferrari, Romina Monzani, Antonella Tosco, Federica Rossin, Manuela D’Eletto, Alice Castaldo, Alessandro Luciani, Marco Silano, Gianni Bona, Gian Luigi Marseglia, Luigina Romani, Mauro Piacentini, Valeria Raia, Guido Kroemer & Luigi Maiuri. (2019) Genistein antagonizes gliadin-induced CFTR malfunction in models of celiac disease. Aging 11:7, pages 2003-2019.
Crossref
Valeria R. Villella, Antonella Tosco, Speranza Esposito, Gianni Bona, Valeria Raia & Luigi Maiuri. (2019) Mutation-specific therapies and drug repositioning in cystic fibrosis. Minerva Pediatrica 71:3.
Crossref
Maria Chiara Maiuri & Guido Kroemer. (2019) Therapeutic modulation of autophagy: which disease comes first?. Cell Death & Differentiation 26:4, pages 680-689.
Crossref
Valeria R. Villella, Speranza Esposito, Eleonora Ferrari, Romina Monzani, Antonella Tosco, Federica Rossin, Alice Castaldo, Marco Silano, Gian Luigi Marseglia, Luigina Romani, Nikolai A. Barlev, Mauro Piacentini, Valeria Raia, Guido Kroemer & Luigi Maiuri. (2019) Autophagy suppresses the pathogenic immune response to dietary antigens in cystic fibrosis. Cell Death & Disease 10:4.
Crossref
Shaoyi Zhang, Yan Wang, Wei Xie, Ethan N. W. Howe, Nathalie Busschaert, Allan Sauvat, Marion Leduc, Lígia C. Gomes-da-Silva, Guo Chen, Isabelle Martins, Xiaxing Deng, Luigi Maiuri, Oliver Kepp, Thierry Soussi, Philip A. Gale, Naoufal Zamzami & Guido Kroemer. (2019) Squaramide-based synthetic chloride transporters activate TFEB but block autophagic flux. Cell Death & Disease 10:3.
Crossref
Mauro Piacentini & Guido Kroemer. (2019) Luigi Maiuri: un Grande Uomo - a Great Spirit. Cell Death & Disease 10:3.
Crossref
Nikhil T. Awatade, Sofia Ramalho, Iris A.L. Silva, Verónica Felício, Hugo M. Botelho, Eyleen de Poel, Annelotte Vonk, Jeffrey M. Beekman, Carlos M. Farinha & Margarida D. Amaral. (2019) R560S: A class II CFTR mutation that is not rescued by current modulators. Journal of Cystic Fibrosis 18:2, pages 182-189.
Crossref
Binyu Lu, Li Li, Molly Schneider, Craig A. Hodges, Calvin U. Cotton, James D. Burgess & Thomas J. Kelley. (2019) Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent. Journal of Cystic Fibrosis 18:2, pages 175-181.
Crossref
Luigi Maiuri, Valeria R Villella, Mauro Piacentini, Valeria Raia & Guido Kroemer. (2019) Defective proteostasis in celiac disease as a new therapeutic target. Cell Death & Disease 10:2.
Crossref
Manish Bodas & Neeraj Vij. (2019) Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease. Frontiers in Pharmacology 10.
Crossref
Francesca GiampieriSadia AfrinTamara Y. Forbes-HernandezMassimiliano GasparriniDanila CianciosiPatricia Reboredo-RodriguezAlfonso Varela-LopezJose L. QuilesMaurizio Battino. (2019) Autophagy in Human Health and Disease: Novel Therapeutic Opportunities. Antioxidants & Redox Signaling 30:4, pages 577-634.
Crossref
Manish Bodas, Garrett Pehote, David Silverberg, Erich Gulbins & Neeraj Vij. (2019) Autophagy augmentation alleviates cigarette smoke-induced CFTR-dysfunction, ceramide-accumulation and COPD-emphysema pathogenesis. Free Radical Biology and Medicine 131, pages 81-97.
Crossref
Mélissa Glatigny, Stéphanie Moriceau, Manon Rivagorda, Mariana Ramos-Brossier, Anna C. Nascimbeni, Fabien Lante, Mary R. Shanley, Nadir Boudarene, Audrey Rousseaud, Allyson K. Friedman, Carmine Settembre, Nicolas Kuperwasser, Gérard Friedlander, Alain Buisson, Etienne Morel, Patrice Codogno & Franck Oury. (2019) Autophagy Is Required for Memory Formation and Reverses Age-Related Memory Decline. Current Biology 29:3, pages 435-448.e8.
Crossref
Valeria R Villella, Andrea Venerando, Giorgio Cozza, Speranza Esposito, Eleonora Ferrari, Romina Monzani, Mara C Spinella, Vasilis Oikonomou, Giorgia Renga, Antonella Tosco, Federica Rossin, Stefano Guido, Marco Silano, Enrico Garaci, Yu‐Kai Chao, Christian Grimm, Alessandro Luciani, Luigina Romani, Mauro Piacentini, Valeria Raia, Guido Kroemer & Luigi Maiuri. (2018) A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease. The EMBO Journal 38:2.
Crossref
Scott M. O’Grady. (2019) Oxidative stress, autophagy and airway ion transport. American Journal of Physiology-Cell Physiology 316:1, pages C16-C32.
Crossref
Manish Bodas & Neeraj Vij. 2019. Oxidative Stress in Lung Diseases. Oxidative Stress in Lung Diseases 389 425 .
Ángel Gabriel Valdivieso. 2019. Oxidative Stress in Lung Diseases. Oxidative Stress in Lung Diseases 355 371 .
Luigi Maiuri & Guido Kroemer. (2018) Autophagy delays progression of the two most frequent human monogenetic lethal diseases: cystic fibrosis and Wilson disease. Aging 10:12, pages 3657-3661.
Crossref
Christian Borgo, Jordi Vilardell, Valentina Bosello-Travain, Lorenzo A. Pinna, Andrea Venerando & Mauro Salvi. (2018) Dependence of HSP27 cellular level on protein kinase CK2 discloses novel therapeutic strategies. Biochimica et Biophysica Acta (BBA) - General Subjects 1862:12, pages 2902-2910.
Crossref
Heon Yung Gee, Jiyoon Kim & Min Goo Lee. (2018) Unconventional secretion of transmembrane proteins. Seminars in Cell & Developmental Biology 83, pages 59-66.
Crossref
Darren M. Hutt, Sanjay Kumar Mishra, Daniela Martino Roth, Mads Breum Larsen, Frédéric Angles, Raymond A. Frizzell & William E. Balch. (2018) Silencing of the Hsp70-specific nucleotide-exchange factor BAG3 corrects the F508del-CFTR variant by restoring autophagy. Journal of Biological Chemistry 293:35, pages 13682-13695.
Crossref
Peter Donald Hurley, Seb Oliver & Anil Mehta. (2018) Creating longitudinal datasets and cleaning existing data identifiers in a cystic fibrosis registry using a novel Bayesian probabilistic approach from astronomy. PLOS ONE 13:7, pages e0199815.
Crossref
Federica Rossin, Valeria Rachela Villella, Manuela D'Eletto, Maria Grazia Farrace, Speranza Esposito, Eleonora Ferrari, Romina Monzani, Luca Occhigrossi, Vittoria Pagliarini, Claudio Sette, Giorgio Cozza, Nikolai A Barlev, Laura Falasca, Gian Maria Fimia, Guido Kroemer, Valeria Raia, Luigi Maiuri & Mauro Piacentini. (2018) TG2 regulates the heat‐shock response by the post‐translational modification of HSF1. EMBO reports 19:7.
Crossref
Hongyu Li, Emanuela Pesce, David N. Sheppard, Ashvani K. Singh & Nicoletta Pedemonte. (2018) Therapeutic approaches to CFTR dysfunction: From discovery to drug development. Journal of Cystic Fibrosis 17:2, pages S14-S21.
Crossref
Valeria Tomati, Emanuela Caci, Loretta Ferrera, Emanuela Pesce, Elvira Sondo, Deborah M. Cholon, Nancy L. Quinney, Susan E. Boyles, Andrea Armirotti, Roberto Ravazzolo, Luis J.V. Galietta, Martina Gentzsch & Nicoletta Pedemonte. (2018) Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia. JCI Insight 3:3.
Crossref
Shaoyi Zhang, Gautier Stoll, José Manuel Bravo San Pedro, Valentina Sica, Allan Sauvat, Florine Obrist, Oliver Kepp, Yousheng Li, Luigi Maiuri, Naoufal Zamzami & Guido Kroemer. (2018) Evaluation of autophagy inducers in epithelial cells carrying the ΔF508 mutation of the cystic fibrosis transmembrane conductance regulator CFTR. Cell Death & Disease 9:2.
Crossref
Neeraj Vij, Prashanth Chandramani-Shivalingappa, Colin Van Westphal, Rachel Hole & Manish Bodas. (2018) Cigarette smoke-induced autophagy impairment accelerates lung aging, COPD-emphysema exacerbations and pathogenesis. American Journal of Physiology-Cell Physiology 314:1, pages C73-C87.
Crossref
Manish Bodas & Neeraj Vij. (2017) Augmenting autophagy for prognosis based intervention of COPD-pathophysiology. Respiratory Research 18:1.
Crossref
Luigi Maiuri, Valeria Raia & Guido Kroemer. (2017) Strategies for the etiological therapy of cystic fibrosis. Cell Death & Differentiation 24:11, pages 1825-1844.
Crossref
Jennifer Guimbellot, Jyoti Sharma & Steven M. Rowe. (2017) Toward inclusive therapy with CFTR modulators: Progress and challenges. Pediatric Pulmonology 52:S48, pages S4-S14.
Crossref
Chandra L. Shrestha, Kaivon D. Assani, Hannah Rinehardt, Florentina Albastroiu, Shuzhong Zhang, Richard Shell, Amal O. Amer, Larry S. Schlesinger & Benjamin T. Kopp. (2017) Cysteamine-mediated clearance of antibiotic-resistant pathogens in human cystic fibrosis macrophages. PLOS ONE 12:10, pages e0186169.
Crossref
Yeon-Ju Lee, Se-Hui Jung, JongYun Hwang, Sohee Jeon, Eun-Taek Han, Won Sun Park, Seok-Ho Hong, Young-Myeong Kim & Kwon-Soo Ha. (2017) Cysteamine prevents vascular leakage through inhibiting transglutaminase in diabetic retina. Journal of Endocrinology 235:1, pages 39-48.
Crossref
Scott Mackenzie Brockman, Manish Bodas, David Silverberg, Ajit Sharma & Neeraj Vij. (2017) Dendrimer-based selective autophagy-induction rescues ΔF508-CFTR and inhibits Pseudomonas aeruginosa infection in cystic fibrosis. PLOS ONE 12:9, pages e0184793.
Crossref
Manish BodasDavid SilverbergKyla WalworthKathryn BruciaNeeraj Vij. (2017) Augmentation of S-Nitrosoglutathione Controls Cigarette Smoke-Induced Inflammatory–Oxidative Stress and Chronic Obstructive Pulmonary Disease-Emphysema Pathogenesis by Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function. Antioxidants & Redox Signaling 27:7, pages 433-451.
Crossref
R. Reilly, M. S. Mroz, E. Dempsey, K. Wynne, S. J. Keely, E. F. McKone, C. Hiebel, C. Behl & J. A. Coppinger. (2017) Targeting the PI3K/Akt/mTOR signalling pathway in Cystic Fibrosis. Scientific Reports 7:1.
Crossref
Daniel L. Hamilos. 2017. The Nose and Sinuses in Respiratory Disorders. The Nose and Sinuses in Respiratory Disorders 48 66 .
Luigina Romani, Vasilis Oikonomou, Silvia Moretti, Rossana G Iannitti, Maria Cristina D'Adamo, Valeria R Villella, Marilena Pariano, Luigi Sforna, Monica Borghi, Marina M Bellet, Francesca Fallarino, Maria Teresa Pallotta, Giuseppe Servillo, Eleonora Ferrari, Paolo Puccetti, Guido Kroemer, Mauro Pessia, Luigi Maiuri, Allan L Goldstein & Enrico Garaci. (2017) Thymosin α1 represents a potential potent single-molecule-based therapy for cystic fibrosis. Nature Medicine 23:5, pages 590-600.
Crossref
Leslie Matalonga, Laura Gort & Antonia Ribes. (2016) Small molecules as therapeutic agents for inborn errors of metabolism. Journal of Inherited Metabolic Disease 40:2, pages 177-193.
Crossref
Giorgio Cozza. (2017) The Development of CK2 Inhibitors: From Traditional Pharmacology to in Silico Rational Drug Design. Pharmaceuticals 10:4, pages 26.
Crossref
Minsoo Song, Hayoung Hwang, Chun Young Im & Soo-Youl Kim. (2016) Recent Progress in the Development of Transglutaminase 2 (TGase2) Inhibitors. Journal of Medicinal Chemistry 60:2, pages 554-567.
Crossref
Eleonora Ferrari, Romina Monzani, Valeria R Villella, Speranza Esposito, Francesca Saluzzo, Federica Rossin, Manuela D'Eletto, Antonella Tosco, Fabiola De Gregorio, Valentina Izzo, Maria C Maiuri, Guido Kroemer, Valeria Raia & Luigi Maiuri. (2017) Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation. Cell Death & Disease 8:1, pages e2544-e2544.
Crossref
Chi B. Vu, Robert J. Bridges, Cecilia Pena-Rasgado, Antonio E. Lacerda, Curtis Bordwell, Abby Sewell, Andrew J. Nichols, Sachin Chandran, Pallavi Lonkar, Dominic Picarella, Amal Ting, Allison Wensley, Maisy Yeager & Feng Liu. (2016) Fatty Acid Cysteamine Conjugates as Novel and Potent Autophagy Activators That Enhance the Correction of Misfolded F508del-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Journal of Medicinal Chemistry 60:1, pages 458-473.
Crossref
Estelle Cormet-Boyaka, Kyle Caution, Duaa Dakhlallah & Amal O. Amer. 2017. Autophagy: Cancer, Other Pathologies, Inflammation, Immunity, Infection, and Aging. Autophagy: Cancer, Other Pathologies, Inflammation, Immunity, Infection, and Aging 139 147 .
Carlo Castellani & Baroukh M. Assael. (2016) Cystic fibrosis: a clinical view. Cellular and Molecular Life Sciences 74:1, pages 129-140.
Crossref
Olga Zegarra-Moran & Luis J. V. Galietta. (2016) CFTR pharmacology. Cellular and Molecular Life Sciences 74:1, pages 117-128.
Crossref
Adam Johnson & Ming Wu. (2016) The New Role for an Old Kinase: Protein Kinase CK2 Regulates Metal Ion Transport. Pharmaceuticals 9:4, pages 80.
Crossref
Speranza Esposito, Antonella Tosco, Valeria R. Villella, Valeria Raia, Guido Kroemer & Luigi Maiuri. (2016) Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis. Molecular and Cellular Pediatrics 3:1.
Crossref
Kiichi Nakahira, Maria Angelica Pabon Porras & Augustine M. K. Choi. (2016) Autophagy in Pulmonary Diseases. American Journal of Respiratory and Critical Care Medicine 194:10, pages 1196-1207.
Crossref
Mieke VeltmanMarta StolarczykDanuta RadziochGabriella WojewodkaJuan B. De SanctisWillem A. DikOleh DzyubachykTamas OraveczIsmé de Kleer & Bob J. Scholte. (2016) Correction of lung inflammation in a F508del CFTR murine cystic fibrosis model by the sphingosine-1-phosphate lyase inhibitor LX2931. American Journal of Physiology-Lung Cellular and Molecular Physiology 311:5, pages L1000-L1014.
Crossref
Samuel Nyabam, Zhuo Wang, Thomas Thibault, Ayinde Oluseyi, Rameeza Basar, Lindsay Marshall & Martin Griffin. (2016) A novel regulatory role for tissue transglutaminase in epithelial-mesenchymal transition in cystic fibrosis. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research 1863:9, pages 2234-2244.
Crossref
Yeon-Ju Lee, Se-Hui Jung, Su-Hyeon Kim, Min-Soo Kim, Sungeun Lee, JongYun Hwang, Soo-Youl Kim, Young-Myeong Kim & Kwon-Soo Ha. (2016) Essential Role of Transglutaminase 2 in Vascular Endothelial Growth Factor–Induced Vascular Leakage in the Retina of Diabetic Mice. Diabetes 65:8, pages 2414-2428.
Crossref
A Tosco, F De Gregorio, S Esposito, D De Stefano, I Sana, E Ferrari, A Sepe, L Salvadori, P Buonpensiero, A Di Pasqua, R Grassia, C A Leone, S Guido, G De Rosa, S Lusa, G Bona, G Stoll, M C Maiuri, A Mehta, G Kroemer, L Maiuri & V Raia. (2016) A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR. Cell Death & Differentiation 23:8, pages 1380-1393.
Crossref
Manish Bodas, Colin Van Westphal, Rhett Carpenter-Thompson, Dillip K. Mohanty & Neeraj Vij. (2016) Nicotine exposure induces bronchial epithelial cell apoptosis and senescence via ROS mediated autophagy-impairment. Free Radical Biology and Medicine 97, pages 441-453.
Crossref
Kris De Boeck & Margarida D Amaral. (2016) Progress in therapies for cystic fibrosis. The Lancet Respiratory Medicine 4:8, pages 662-674.
Crossref
Graham Devereux, Sandra Steele, Kairen Griffiths, Edward Devlin, Douglas Fraser-Pitt, Seonaidh Cotton, John Norrie, Henry Chrystyn & Deborah O?Neil. (2016) An Open-Label Investigation of the Pharmacokinetics and Tolerability of Oral Cysteamine in Adults with Cystic Fibrosis. Clinical Drug Investigation 36:8, pages 605-612.
Crossref
Guido Veit, Kathryn Oliver, Pirjo M. Apaja, Doranda Perdomo, Aurélien Bidaud-Meynard, Sheng-Ting Lin, Jingyu Guo, Mert Icyuz, Eric J. Sorscher, John L. Hartman & Gergely L. Lukacs. (2016) Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect. PLOS Biology 14:5, pages e1002462.
Crossref
Jiang Li, Ting‑Ting Zhao, Peng Zhang, Chao‑Jin Xu, Zhuo‑Xiang Rong, Zi‑Yi Yan & Chang‑Yun Fang. (2016) Autophagy mediates oral submucous fibrosis. Experimental and Therapeutic Medicine.
Crossref
Prashanth Chandramani ShivalingappaRachel HoleColin Van WestphalNeeraj Vij. (2016) Airway Exposure to E-Cigarette Vapors Impairs Autophagy and Induces Aggresome Formation. Antioxidants & Redox Signaling 24:4, pages 186-204.
Crossref
Lisa Frost, Pratap Suryadevara, Stephanie J. Cannell, Paul W. Groundwater, Paul A. Hambleton & Rosaleen J. Anderson. (2016) Synthesis of diacylated γ-glutamyl-cysteamine prodrugs, and in vitro evaluation of their cytotoxicity and intracellular delivery of cysteamine. European Journal of Medicinal Chemistry 109, pages 206-215.
Crossref
Carlos M. Farinha, Agnieszka Swiatecka-Urban, David L. Brautigan & Peter Jordan. (2016) Regulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity. Frontiers in Chemistry 4.
Crossref
Mariana Maciel-Herrerías & Sandra Cabrera-Benítez. (2016) El papel de la autofagia en enfermedades pulmonares. NCT Neumología y Cirugía de Tórax 75:3, pages 227-236.
Crossref
Carlos M. Farinha & Paulo Matos. (2016) Repairing the basic defect in cystic fibrosis - one approach is not enough. FEBS Journal 283:2, pages 246-264.
Crossref
A. A. Zeki, B. Yeganeh, N. J. Kenyon, M. Post & S. Ghavami. (2016) Autophagy in airway diseases: a new frontier in human asthma?. Allergy 71:1, pages 5-14.
Crossref
Mohammad S. Zaman, Adam J. Johnson, Gabriele Bobek, Sindy Kueh, Cindy Kersaitis, Trevor D. Bailey, Yossi Buskila & Ming J. Wu. (2016) Protein kinase CK2 regulates metal toxicity in neuronal cells. Metallomics 8:1, pages 82-90.
Crossref
Barbara Bosch & Kris De Boeck. (2015) Searching for a cure for cystic fibrosis. A 25-year quest in a nutshell. European Journal of Pediatrics 175:1, pages 1-8.
Crossref
Margarida D. Amaral & William E. Balch. (2015) Hallmarks of therapeutic management of the cystic fibrosis functional landscape. Journal of Cystic Fibrosis 14:6, pages 687-699.
Crossref
Daniela De Stefano & Maria Chiara Maiuri. (2015) A Breathe in Cystic Fibrosis Therapy: A New Therapeutic Endeavor for Cysteamine. EBioMedicine 2:10, pages 1306-1307.
Crossref
Graham Devereux, Douglas Fraser-Pitt, Jennifer Robertson, Edward Devlin, Derry Mercer & Deborah O'Neil. (2015) Cysteamine as a Future Intervention in Cystic Fibrosis Against Current and Emerging Pathogens: A Patient-based ex vivo Study Confirming its Antimicrobial and Mucoactive Potential in Sputum. EBioMedicine 2:10, pages 1507-1512.
Crossref
Marisa Ponpuak, Michael A Mandell, Tomonori Kimura, Santosh Chauhan, C?dric Cleyrat & Vojo Deretic. (2015) Secretory autophagy. Current Opinion in Cell Biology 35, pages 106-116.
Crossref
Stefan W. Ryter & Augustine M.K. Choi. (2015) Autophagy in lung disease pathogenesis and therapeutics. Redox Biology 4, pages 215-225.
Crossref
Kevin Nishida, Zachary Smith, Dane Rana, Jereme Palmer & G. Ian Gallicano. (2015) Cystic fibrosis: A look into the future of prenatal screening and therapy. Birth Defects Research Part C: Embryo Today: Reviews 105:1, pages 73-80.
Crossref
Guido Kroemer. (2015) Autophagy: a druggable process that is deregulated in aging and human disease. Journal of Clinical Investigation 125:1, pages 1-4.
Crossref
Manuela D’Eletto, Federica Rossin, Maria Grazia Farrace & Mauro Piacentini. 2015. Transglutaminases. Transglutaminases 171 191 .

Related research

People also read lists articles that other readers of this article have read.

Recommended articles lists articles that we recommend and is powered by our AI driven recommendation engine.

Cited by lists all citing articles based on Crossref citations.
Articles with the Crossref icon will open in a new tab.