المؤلفون: Liberski S; Department of Ophthalmology, Poznan University of Medical Sciences, A. Szamarzewskiego 84, 61-848 Poznan, Poland., Confalonieri F; Department of Ophthalmology, IRCCS Humanitas Research Hospital, 20089 Rozzano, Milan, Italy.; Department of Biomedical Sciences, Humanitas University, 20090 Pieve Emanuele, Milan, Italy.; Center for Eye Research and Innovative Diagnostics, Department of Ophthalmology, Institute for Clinical Medicine, University of Oslo, Kirkeveien 166, 0450 Oslo, Norway.; Department of Ophthalmology, Oslo University Hospital, Kirkeveien 166, 0450 Oslo, Norway., Cofta S; Department of Respiratory Medicine, Allergology and Pulmonary Oncology, Poznan University of Medical Sciences, A. Szamarzewskiego 84, 61-848 Poznan, Poland., Petrovski G; Center for Eye Research and Innovative Diagnostics, Department of Ophthalmology, Institute for Clinical Medicine, University of Oslo, Kirkeveien 166, 0450 Oslo, Norway.; Department of Ophthalmology, Oslo University Hospital, Kirkeveien 166, 0450 Oslo, Norway., Kocięcki J; Department of Ophthalmology, Poznan University of Medical Sciences, A. Szamarzewskiego 84, 61-848 Poznan, Poland.

المصدر: International journal of molecular sciences [Int J Mol Sci] 2024 Jun 18; Vol. 25 (12). Date of Electronic Publication: 2024 Jun 18.

نوع المنشور: Journal Article; Review

بيانات الدورية: Publisher: MDPI Country of Publication: Switzerland NLM ID: 101092791 Publication Model: Electronic Cited Medium: Internet ISSN: 1422-0067 (Electronic) Linking ISSN: 14220067 NLM ISO Abbreviation: Int J Mol Sci Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis*/metabolism , Cystic Fibrosis*/genetics , Cystic Fibrosis*/pathology , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism, Humans ; Eye Diseases/etiology ; Eye Diseases/metabolism ; Eye Diseases/pathology ; Mutation ; Animals

عنوان ترانسليتريتد: Mukoviszidose im Kindes- und im Erwachsenenalter.

المؤلفون: Syunyaeva Z; Klinik für Pädiatrie m. S. Pneumologie, Immunologie und Intensivmedizin, Sektion Mukoviszidose, Campus Virchow Klinikum, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland. Zulfiya.Syunyaeva@charite.de., Mall MA; Klinik für Pädiatrie m. S. Pneumologie, Immunologie und Intensivmedizin, Sektion Mukoviszidose, Campus Virchow Klinikum, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland.; Deutsches Zentrum für Lungenforschung (DZL), assoziierter Partnerstandort, Berlin, Deutschland.; Berlin Institute of Health (BIH), Charité - Universitätsmedizin Berlin, Berlin, Deutschland., Stahl M; Klinik für Pädiatrie m. S. Pneumologie, Immunologie und Intensivmedizin, Sektion Mukoviszidose, Campus Virchow Klinikum, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland.; Deutsches Zentrum für Lungenforschung (DZL), assoziierter Partnerstandort, Berlin, Deutschland.; Berlin Institute of Health (BIH), Charité - Universitätsmedizin Berlin, Berlin, Deutschland.

المصدر: Innere Medizin (Heidelberg, Germany) [Inn Med (Heidelb)] 2024 Jun; Vol. 65 (6), pp. 538-544. Date of Electronic Publication: 2024 May 07.

نوع المنشور: Journal Article; Review

بيانات الدورية: Publisher: Springer Medizin Country of Publication: Germany NLM ID: 9918384885306676 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2731-7099 (Electronic) Linking ISSN: 27317080 NLM ISO Abbreviation: Inn Med (Heidelb) Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis*/genetics , Cystic Fibrosis*/therapy , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism, Humans ; Child ; Adult ; Infant, Newborn ; Adolescent ; Neonatal Screening ; Prognosis ; Aminophenols/therapeutic use ; Quality of Life

المؤلفون: Eldredge JA; Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia. Electronic address: jessica.eldredge@rch.org.au., Oliver MR; Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Australia. Electronic address: mark.oliver@rch.org.au., Ooi CY; Department of Gastroenterology, Sydney Children's Hospital Randwick, NSW, Australia; School of Clinical Medicine, Discipline of Paediatrics and Child Health, UNSW Medicine & Health, University of New South Wales, Sydney, Australia. Electronic address: keith.ooi@unsw.edu.au.

المصدر: Paediatric respiratory reviews [Paediatr Respir Rev] 2024 Jun; Vol. 50, pp. 54-61. Date of Electronic Publication: 2023 Dec 28.

نوع المنشور: Journal Article; Review

بيانات الدورية: Publisher: W.B. Saunders Country of Publication: England NLM ID: 100898941 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1526-0550 (Electronic) Linking ISSN: 15260542 NLM ISO Abbreviation: Paediatr Respir Rev Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/complications , Cystic Fibrosis*/physiopathology , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Liver Diseases*/metabolism , Liver Diseases*/etiology, Humans ; Liver Transplantation ; Benzodioxoles/therapeutic use ; Aminophenols/therapeutic use ; Quinolones/therapeutic use ; Aminopyridines/therapeutic use ; Pyrazoles/therapeutic use

المؤلفون: Leir SH; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA., Tkachenko S; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA., Paranjapye A; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA.; Department of Genetics, University of Pennsylvania, Philadelphia, PA, USA., Meckler F; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA., Van Wettere AJ; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., Kerschner JL; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA., Kuznetsov E; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA., Schacht M; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA., Gillurkar P; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA., Regouski M; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., Viotti Perisse I; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., Marriott CM; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., Liu Y; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., Bunderson I; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., White KL; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., Polejaeva IA; Department of Animal, Dairy and Veterinary Sciences, Utah State University, Logan, UT, USA., Harris A; Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106-4955, USA. ann.harris@case.edu.

المصدر: Molecular medicine (Cambridge, Mass.) [Mol Med] 2024 Aug 07; Vol. 30 (1), pp. 115. Date of Electronic Publication: 2024 Aug 07.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: BioMed Central Country of Publication: England NLM ID: 9501023 Publication Model: Electronic Cited Medium: Internet ISSN: 1528-3658 (Electronic) Linking ISSN: 10761551 NLM ISO Abbreviation: Mol Med Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis*/genetics , Cystic Fibrosis*/metabolism , Cystic Fibrosis*/pathology , Disease Models, Animal* , Pancreatic Stellate Cells*/metabolism , Pancreatic Stellate Cells*/pathology , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Biomarkers*, Animals ; Female ; Sheep ; Pancreas/metabolism ; Pancreas/pathology ; Pregnancy ; Pancreatic Diseases/genetics ; Pancreatic Diseases/metabolism ; Pancreatic Diseases/pathology ; Transcriptome ; Humans ; Gene Expression Profiling

المؤلفون: Gao X; Dalton Cardiovascular Research Center, University of Missouri-Columbia, Columbia, MO, 65211, USA. xgdz2@missouri.edu., Yeh HI; Dalton Cardiovascular Research Center, University of Missouri-Columbia, Columbia, MO, 65211, USA.; Institute of Pharmacology, National Yang Ming Chiao Tung University, College of Medicine, Taipei, Taiwan.; Membrane Protein Structural Biology Research Center, National Yang Ming Chiao Tung University, Taipei, Taiwan., Yang Z; Heersink School of Medicine, University of Alabama School of Medicine, Birmingham, AL, 35233, USA., Fan C; Department of Applied Physics, Science for Life Laboratory, KTH Royal Institute of Technology, Solna, Sweden.; Department of Biochemistry and Biophysics, Science for Life Laboratory, Stockholm University, Solna, Sweden., Jiang F; Heersink School of Medicine, University of Alabama School of Medicine, Birmingham, AL, 35233, USA., Howard RJ; Department of Applied Physics, Science for Life Laboratory, KTH Royal Institute of Technology, Solna, Sweden.; Department of Biochemistry and Biophysics, Science for Life Laboratory, Stockholm University, Solna, Sweden., Lindahl E; Department of Applied Physics, Science for Life Laboratory, KTH Royal Institute of Technology, Solna, Sweden.; Department of Biochemistry and Biophysics, Science for Life Laboratory, Stockholm University, Solna, Sweden., Kappes JC; Heersink School of Medicine, University of Alabama School of Medicine, Birmingham, AL, 35233, USA.; Research Service, Birmingham Veterans Affairs Medical Center, Veterans Health Administration, Birmingham, AL, 35233, USA., Hwang TC; Dalton Cardiovascular Research Center, University of Missouri-Columbia, Columbia, MO, 65211, USA. hwangt@health.missouri.edu.; Institute of Pharmacology, National Yang Ming Chiao Tung University, College of Medicine, Taipei, Taiwan. hwangt@health.missouri.edu.; Membrane Protein Structural Biology Research Center, National Yang Ming Chiao Tung University, Taipei, Taiwan. hwangt@health.missouri.edu.

المصدر: Nature communications [Nat Commun] 2024 Aug 06; Vol. 15 (1), pp. 6668. Date of Electronic Publication: 2024 Aug 06.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: Nature Pub. Group Country of Publication: England NLM ID: 101528555 Publication Model: Electronic Cited Medium: Internet ISSN: 2041-1723 (Electronic) Linking ISSN: 20411723 NLM ISO Abbreviation: Nat Commun Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator*/chemistry , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cryoelectron Microscopy*, Humans ; Allosteric Regulation ; Ion Channel Gating/drug effects ; Cystic Fibrosis/metabolism ; Cystic Fibrosis/genetics ; Cystic Fibrosis/drug therapy ; Animals ; Protein Binding ; Aminophenols/pharmacology ; Aminophenols/chemistry ; Aminophenols/metabolism ; Benzodioxoles/pharmacology ; Mutation

المؤلفون: Zhang J; Department of Gastroenterology, The Affiliated Ganzhou Hospital of Nanchang University, Ganzhou City, China., Liu X; Department of Gastroenterology, The Affiliated Ganzhou Hospital of Nanchang University, Ganzhou City, China., Zeng L; Department of Gastroenterology, The Affiliated Ganzhou Hospital of Nanchang University, Ganzhou City, China., Hu Y; Department of Gastroenterology, The First Affiliated Hospital of Gannan Medical University, Ganzhou City, China.

المصدر: International journal of experimental pathology [Int J Exp Pathol] 2024 Aug; Vol. 105 (4), pp. 118-132. Date of Electronic Publication: 2024 Jul 11.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: Wiley Country of Publication: England NLM ID: 9014042 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1365-2613 (Electronic) Linking ISSN: 09599673 NLM ISO Abbreviation: Int J Exp Pathol Subsets: MEDLINE

مواضيع طبية MeSH: Esophageal Neoplasms*/genetics , Esophageal Neoplasms*/pathology , Esophageal Neoplasms*/metabolism , Epithelial-Mesenchymal Transition*/genetics , Gene Expression Regulation, Neoplastic* , Cell Proliferation* , Cell Movement* , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Computational Biology*/methods , Kruppel-Like Factor 4*/metabolism, Humans ; Cell Line, Tumor ; Adaptor Proteins, Signal Transducing/genetics ; Adaptor Proteins, Signal Transducing/metabolism ; Disease Progression ; Neoplasm Invasiveness ; Microtubule-Associated Proteins ; Apoptosis Regulatory Proteins

المؤلفون: Crunkhorn S

المصدر: Nature reviews. Drug discovery [Nat Rev Drug Discov] 2024 Aug; Vol. 23 (8), pp. 581.

نوع المنشور: Journal Article; News; Comment

بيانات الدورية: Publisher: Nature Pub. Group Country of Publication: England NLM ID: 101124171 Publication Model: Print Cited Medium: Internet ISSN: 1474-1784 (Electronic) Linking ISSN: 14741776 NLM ISO Abbreviation: Nat Rev Drug Discov Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics, Humans ; Drug Discovery ; Animals

المؤلفون: Ljungholm PL; Department of Physiology, University of Gothenburg, Medicinaregatan 11, Box 432, 405 30, Gothenburg, Sweden., Ermund A; Department of Medical Chemistry and Cell Biology, University of Gothenburg, Gothenburg, Sweden., Söderlund Garsveden MM; Department of Physiology, University of Gothenburg, Medicinaregatan 11, Box 432, 405 30, Gothenburg, Sweden., Pettersson VL; Department of Physiology, University of Gothenburg, Medicinaregatan 11, Box 432, 405 30, Gothenburg, Sweden., Gustafsson JK; Department of Physiology, University of Gothenburg, Medicinaregatan 11, Box 432, 405 30, Gothenburg, Sweden. jenny.gustafsson@gu.se.; Department of Medical Chemistry and Cell Biology, University of Gothenburg, Gothenburg, Sweden. jenny.gustafsson@gu.se.

المصدر: Pflugers Archiv : European journal of physiology [Pflugers Arch] 2024 Aug; Vol. 476 (8), pp. 1209-1219. Date of Electronic Publication: 2024 Jun 03.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: Springer Country of Publication: Germany NLM ID: 0154720 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-2013 (Electronic) Linking ISSN: 00316768 NLM ISO Abbreviation: Pflugers Arch Subsets: MEDLINE

مواضيع طبية MeSH: Dinoprostone*/metabolism , Dinoprostone*/pharmacology , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Sulfate Transporters*/metabolism , Sulfate Transporters*/genetics , Colon*/metabolism , Colon*/drug effects , Mucus*/metabolism , Mucus*/drug effects , Carbachol*/pharmacology, Animals ; Mice ; Mice, Inbred C57BL ; 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid/pharmacology ; Antiporters/metabolism ; Intestinal Mucosa/metabolism ; Intestinal Mucosa/drug effects ; Male

المؤلفون: Meng X; University of Manchester, School of Biological Sciences, Oxford Road, Manchester, M13 9PL, UK; The Francis Crick Institute, Cellular Degradation Systems Lab, 1 Midland Road, London, NW1 1AT, UK., Ford RC; University of Manchester, School of Biological Sciences, Oxford Road, Manchester, M13 9PL, UK. Electronic address: robert.ford@manchester.ac.uk.

المصدر: Archives of biochemistry and biophysics [Arch Biochem Biophys] 2024 Aug; Vol. 758, pp. 110050. Date of Electronic Publication: 2024 Jun 12.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: Elsevier Country of Publication: United States NLM ID: 0372430 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1096-0384 (Electronic) Linking ISSN: 00039861 NLM ISO Abbreviation: Arch Biochem Biophys Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis Transmembrane Conductance Regulator*/chemistry , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Small Molecule Libraries*/pharmacology , Small Molecule Libraries*/chemistry, Humans ; Kinetics ; Protein Unfolding ; Protein Stability ; Cystic Fibrosis/metabolism ; Cystic Fibrosis/genetics ; Cystic Fibrosis/drug therapy ; Mutation

المؤلفون: Castaldo A; Dipartimento di Scienze Mediche Traslazionali, Sezione di Pediatria, Università di Napoli Federico II, Naples, Italy.; SC di Pneumologia e UTSIR, AORN Santobono-Pausilipon, Naples, Italy.; CEINGE-Biotecnologie Avanzate Franco Salvatore, Naples, Italy.; Dipartimento di Pediatria, Azienda Ospedaliera Universitaria Meyer IRCCS, Centro Regionale Toscano per la cura della Fibrosi Cistica, Florence, Italy., Iacotucci P; Dipartimento di Medicina Clinica e Chirurgia, Università di Napoli Federico II, Naples, Italy., Bagnasco S; CEINGE-Biotecnologie Avanzate Franco Salvatore, Naples, Italy., Fevola C; Dipartimento di Pediatria, Azienda Ospedaliera Universitaria Meyer IRCCS, Centro Regionale Toscano per la cura della Fibrosi Cistica, Florence, Italy., Carnovale V; Dipartimento di Scienze Mediche Traslazionali, Centro di Fibrosi Cistica dell'Adulto, Università di Napoli Federico II, Naples, Italy., Antonelli F; SC di Pneumologia e UTSIR, AORN Santobono-Pausilipon, Naples, Italy., Cernera G; CEINGE-Biotecnologie Avanzate Franco Salvatore, Naples, Italy.; Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli Federico II, Naples, Italy., Gelzo M; CEINGE-Biotecnologie Avanzate Franco Salvatore, Naples, Italy. monica.gelzo@unina.it.; Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli Federico II, Naples, Italy. monica.gelzo@unina.it., Terlizzi V; Dipartimento di Pediatria, Azienda Ospedaliera Universitaria Meyer IRCCS, Centro Regionale Toscano per la cura della Fibrosi Cistica, Florence, Italy.

المصدر: Scientific reports [Sci Rep] 2024 Jul 29; Vol. 14 (1), pp. 17422. Date of Electronic Publication: 2024 Jul 29.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics , Cystic Fibrosis*/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Cholesterol*/metabolism , Cholesterol*/blood , Benzodioxoles*/therapeutic use , Aminophenols*/therapeutic use , Indoles*/therapeutic use , Indoles*/adverse effects , Liver*/metabolism , Liver*/drug effects , Genotype* , Quinolones*/therapeutic use , Quinolones*/adverse effects, Humans ; Female ; Male ; Adult ; Adolescent ; Young Adult ; Pyrazoles/therapeutic use ; Pyrazoles/pharmacology ; Mutation ; Child ; Drug Combinations ; Pyridines/therapeutic use ; Pyridines/administration & dosage ; Pyridines/adverse effects ; Pyrrolidines/therapeutic use ; Pyrrolidines/pharmacology ; Pyrrolidines/administration & dosage