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1Academic Journal
المؤلفون: Saba Anwar, Jin-Liang Peng, Kashif Rafiq Zahid, Yu-Ming Zhou, Qurban Ali, Chong-Rong Qiu
المصدر: Advances in Respiratory Medicine, Vol 92, Iss 4, Pp 263-277 (2024)
مصطلحات موضوعية: cystic fibrosis, fibrosis transmembrane regulator, targeted mutation, pathophysiology, modulators, Diseases of the respiratory system, RC705-779, Medicine (General), R5-920
وصف الملف: electronic resource
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2Academic Journal
المؤلفون: Anwar, Saba, Peng, Jin-Liang, Zahid, Kashif Rafiq, Zhou, Yu-Ming, Ali, Qurban, Qiu, Chong-Rong
المساهمون: Radiation Oncology, School of Medicine
المصدر: PMC
مصطلحات موضوعية: Cystic fibrosis, Fibrosis transmembrane regulator, Targeted mutation, Pathophysiology, Modulators
وصف الملف: application/pdf
Relation: Advances in Respiratory Medicine; Anwar S, Peng JL, Zahid KR, Zhou YM, Ali Q, Qiu CR. Cystic Fibrosis: Understanding Cystic Fibrosis Transmembrane Regulator Mutation Classification and Modulator Therapies. Adv Respir Med. 2024;92(4):263-277. Published 2024 Jul 20. doi:10.3390/arm92040026; https://hdl.handle.net/1805/43323
الاتاحة: https://hdl.handle.net/1805/43323
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3Academic Journal
المؤلفون: E. B. Pavlinova, T. I. Safonova, I. A. Kirshina, D. A. Medvedeva, T. Yu. Korneeva, N. A. Basyukova, A. A. Shevlyakova, Е. Б. Павлинова, Т. И. Сафонова, И. А. Киршина, Д. А. Медведева, Т. Ю. Корнеева, Н. А. Басюкова, А. А. Шевлякова
المصدر: PULMONOLOGIYA; Том 34, № 2 (2024); 231-237 ; Пульмонология; Том 34, № 2 (2024); 231-237 ; 2541-9617 ; 0869-0189
مصطلحات موضوعية: таргетная терапия, CFTR, cystic fibrosis transmembrane regulator modulators, targeted therapy, модуляторы трансмембранного регулятора муковисцидоза
وصف الملف: application/pdf
Relation: https://journal.pulmonology.ru/pulm/article/view/4431/3644; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4431/2449; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4431/2450; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4431/2603; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4431/2604; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4431/2605; Министерство здравоохранения Российской Федерации. Клинические рекомендации: Кистозный фиброз (муковисцидоз). 2021. Доступно на: https://cr.minzdrav.gov.ru/schema/372_2?ysclid=ltpux0qqcj90886013 [Дата обращения: 28.02.24].; Bell S.C., Mall M. A., Gutierrez H. et al. The future of cystic fibrosis care: a global perspective. Lancet Respir. Med. 2020; 8 (1): 65–124. DOI:10.1016/S2213-2600(19)30337-6.; Регистр больных муковисцидозом в Российской Федерации 2011 год. Пульмонология. 2014. Приложение. Доступно на: https://mukoviscidoz.org/doc/registr/Registr_end_2011.pdf?ysclid=ltpv0ooqgk81369922; Кондратьева Е.И., Каширская Н.Ю., Капранов Н.И., ред. Национальный консенсус «Муковисцидоз: определение, диагностические критерии, терапия». 2-е изд. М.; 2019. Доступно на: https://mukoviscidoz.org/doc/konsensus/2019/konsensus-2019-bezrentgenogramm.pdf?ysclid=ltpv5psoun562052220 [Дата обращения: 28.02.24].; Кондратьева Е.И., Кондакова Ю.А., Назаренко Л. П. и др. Клинико-эпидемиологические характеристики больных муковисцидозом, проживающих на территории Сибирского федерального округа, по данным национального регистра (2017). Пульмонология. 2020; 30 (6): 764–775. DOI:10.18093/0869-0189-2020-30-6-764-775.; Шагинян И.А., Чернуха М.Ю., Аветисян Л.Р. и др. Эпидемиологические особенности хронической инфекции легких у больных муковисцидозом. Эпидемиология и вакцинопрофилактика. 2017; 16 (6): 5–13. DOI:10.31631/2073-3046-2017-16-6-5-13.; Clancy J.P., Cotton C.U., Donaldson S.H. et al. CFTR modulator theratyping: current status, gaps and future directions. J. Cyst. Fibros. 2019; 18 (1): 22–34. DOI:10.1016/S2213-2600(19)30337-6.; https://journal.pulmonology.ru/pulm/article/view/4431
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4
المؤلفون: Vanherle, Lotte
المصدر: Lund University, Faculty of Medicine Doctoral Dissertation Series. (2023:109)
مصطلحات موضوعية: Heart Failure, Cystic fibrosis transmembrane regulator, Target organ damage, Brain, Lung, Medicin och hälsovetenskap, Medicinska och farmaceutiska grundvetenskaper, Fysiologi, Medical and Health Sciences, Basic Medicine, Physiology
وصف الملف: electronic
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5Academic Journal
المؤلفون: E. I. Kondratyeva, N. D. Odinaeva, E. K. Zhekaite, E. V. Pasnova, I. R. Fatkhullina, T. Yu. Maksimycheva, V. D. Sherman, S. I. Kutsev, Е. И. Кондратьева, Н. Д. Одинаева, Е. К. Жекайте, Е. В. Паснова, И. Р. Фатхуллина, Т. Ю. Максимычева, В. Д. Шерман, С. И. Куцев
المساهمون: The work was carried out under the state task of the Ministry of Science and Higher Education of the Russian Federation for the Federal State Budgetary Scientific Institution “Research Centre for Medical Genetics”, Ministry of Science and Higher Education of the Russian Federation, on the topic: “Comprehensive analysis of geno-phenotypic correlations in cystic fibrosis and primary ciliary dyskinesia” No.122032300396-1, Работа выполнена в рамках государственного задания Министерства науки и высшего образования Российской Федерации для Федерального государственного бюджетного научного учреждения «Медико-генетический научный центр имени академика Н.П.Бочкова» Министерства науки и высшего образования Российской Федерации по теме «Комплексный анализ генофенотипических корреляций при муковисцидозе и первичной цилиарной дискинезии» № 122032300396-1
المصدر: PULMONOLOGIYA; Том 33, № 2 (2023); 189-197 ; Пульмонология; Том 33, № 2 (2023); 189-197 ; 2541-9617 ; 0869-0189
مصطلحات موضوعية: функция внешнего дыхания, CFTR, modulators of the transmembrane regulator of cystic fibrosis, targeted therapy, sweat test, respiratory function, модуляторы трансмембранного регулятора муковисцидоза, таргетная терапия, потовый тест
وصف الملف: application/pdf
Relation: https://journal.pulmonology.ru/pulm/article/view/4213/3510; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1919; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1920; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1921; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1922; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1923; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1924; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1925; https://journal.pulmonology.ru/pulm/article/downloadSuppFile/4213/1926; Veit G., Avramescu R.G., Chiang A.N. et al. From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol. Biol. Cell. 2016; 27 (3): 424-433. DOI:10.1091/mbc.E14-04-0935.; Koch C., Hoiby N. Pathogenesis of cystic fibrosis. Lancet. 1993; 341 (8852): 1065-1069. DOI:10.1016/0140-6736(93)92422-p.; Cain C. Cystic fibrosis two-step. SciBX. 2012; 5 (8): 192. https://doi.org/10.1038/scibx.2012.192; Куцев С.И., Ижевская В.Л., Кондратьева Е.И. Таргетная терапия при муковисцидозе. Пульмонология. 2021; 31 (2): 226-237. DOI:10.18093/0869-0189-2021-31-2-226-236.; Van Goor F., Hadida S., Grootenhuis P.D. et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc. Natl. Acad. Sci. USA. 2011; 108 (46): 18843-18848. DOI:10.1073/pnas.1105787108.; Southern K.W., Patel S., Sinha I.P., Nevitt S.J. Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis. Cochrane Database Syst. Rev. 2018; 8 (8): CD010966. DOI:10.1002/14651858.cd010966.pub2.; Zaher A., Saygh J.E., Elsori D. et al. A review of Trikafta: triple cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy. Cureus. 2021; 13 (7): e16144. DOI:10.7759/cureus.16144; Кондратьева Е.И., Одинаева Н.Д., Шерман В.Д. и др. Первые результаты терапии двумя CFTR-модуляторами при муковисцидозе в детском возрасте. Педиатрия. Журнал имени Г.Н.Сперанского. 2022; 101 (3): 98-105. DOI:10.24110/0031-403X-2022-101-3-98-105.; Middleton P.G., Mall M.A., Drevinek P. et al. Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single phe508del allele. N. Engl. J. Med. 2019; 381 (19): 1809-1819. https://doi.org/10.1056/NEJMoa1908639; Fajac I., Daines C., Durieu I. et al. Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/ tezacaftor/ivacaftor. J. Cyst. Fibros. 2022; 13: S1569-1993(22)00655-5. DOI:10.1016/j.jcf.2022.08.018.; Минздрав Российской Федерации. Клинические рекомендации: Кистозный фиброз (муковисцидоз). 2021. Доступно на: https://mukoviscidoz.org/doc/%D0%9A%D0%A0372.pdf; Donaldson S.H., Laube B.L., Mogayzel P. et al. Effect of luma-caftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: results from the PROSPECT MCC sub-study. J. Cyst. Fibros. 2022; 21 (1): 143-145. DOI:10.1016/j.jcf.2021.05.004.; Carter S.C., Kearns S., Grogan B. et al. Effects of lumacaftor/iva-caftor in patients homozygous for F508del mutation with very advanced lung disease. J. Cyst. Fibros. 2017; 16 (Suppl. 1): S76. DOI:10.1016/S1569-1993(17)30412-5.; Cai Z.W., Liu J, Li H.Y., Sheppard D.N. Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis. Acta Pharmacol. Sin. 2011; 32 (6): 693-701. DOI:10.1038/aps.2011.71.; Taylor-Cousar J.L., Mall M.A., Ramsey B.W. et al. Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles. ERJ Open Res. 2019; 5 (2): 00082-2019. DOI:10.1183/23120541.00082-2019.; Heijerman H.G., McKone E.F., Downey D.G. et al. Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial. Lancet. 2019; 394 (10212): 1940-1948. DOI:10.1016/S0140-6736(19)32597-8.; https://journal.pulmonology.ru/pulm/article/view/4213
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6Academic Journal
المصدر: Molecules, Vol 28, Iss 6581, p 6581 (2023)
مصطلحات موضوعية: chitosan, epithelial sodium channels, chloride ion transport, cystic fibrosis transmembrane regulator, electric potential, electric resistance, Organic chemistry, QD241-441
Relation: https://www.mdpi.com/1420-3049/28/18/6581; https://doaj.org/toc/1420-3049; https://doaj.org/article/c13e6b32fae844098e17d267b744cb33
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7Academic Journal
المؤلفون: Lotte Vanherle, Frank Matthes, Franziska E. Uhl, Anja Meissner
المصدر: Biomedicine & Pharmacotherapy, Vol 162, Iss , Pp 114628- (2023)
مصطلحات موضوعية: Myocardial infarction, Cystic fibrosis transmembrane regulator, Ivacaftor, Inflammation, Target tissue damage, Therapeutics. Pharmacology, RM1-950
Relation: http://www.sciencedirect.com/science/article/pii/S075333222300416X; https://doaj.org/toc/0753-3322; https://doaj.org/article/7fe8dbd075dc4dd78d3c8ce7e0c2d3a6
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8Academic Journal
المؤلفون: Franziska E. Uhl, Lotte Vanherle, Anja Meissner
المصدر: Frontiers in Immunology, Vol 13 (2022)
مصطلحات موضوعية: lung, heart failure, cystic fibrosis transmembrane regulator, inflammation, macrophages, Immunologic diseases. Allergy, RC581-607
وصف الملف: electronic resource
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9Academic Journal
المؤلفون: Elena K. Schneider-Futschik, Viola H. Lobert, John W. Wilson
المصدر: Frontiers in Physiology, Vol 12 (2022)
مصطلحات موضوعية: CFTR (cystic fibrosis transmembrane conductance regulator), CFTR (cystic fibrosis transmembrane regulator), cystic fibrosis, lung, biomarker, Physiology, QP1-981
وصف الملف: electronic resource
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10Academic Journal
المؤلفون: Julie Mésinèle, Manon Ruffin, Loïc Guillot, Harriet Corvol
المصدر: International Journal of Molecular Sciences; Volume 23; Issue 22; Pages: 14205
مصطلحات موضوعية: cystic fibrosis, modifiers genes, environmental factors, CFTR (Cystic Fibrosis Transmembrane Regulator) modulator therapy
جغرافية الموضوع: agris
وصف الملف: application/pdf
Relation: https://dx.doi.org/10.3390/ijms232214205
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11Academic Journal
المؤلفون: Franziska E. Uhl, Lotte Vanherle, Frank Matthes, Anja Meissner
المصدر: International Journal of Molecular Sciences; Volume 23; Issue 2; Pages: 866
مصطلحات موضوعية: heart failure, sphingosine-1-phosphate, cystic fibrosis transmembrane regulator, inflammation, lung
جغرافية الموضوع: agris
وصف الملف: application/pdf
Relation: Biochemistry; https://dx.doi.org/10.3390/ijms23020866
الاتاحة: https://doi.org/10.3390/ijms23020866
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12Academic Journal
المؤلفون: Ye, Long, Hu, Bao, El-Badri, Faris, Hudson, Brandi M, Phuan, Puay-Wah, Verkman, AS, Tantillo, Dean J, Kurth, Mark J
المصدر: Bioorganic & medicinal chemistry letters. 24(24)
مصطلحات موضوعية: Humans, Cystic Fibrosis, Water, Imidazoles, Oxazoles, Oxadiazoles, Thiazoles, Thiadiazoles, Cystic Fibrosis Transmembrane Conductance Regulator, Molecular Conformation, Protein Binding, Structure-Activity Relationship, Kinetics, Thermodynamics, C, C-linked bisazoles, Correctors, Cystic fibrosis, Transmembrane regulator, Lung, Rare Diseases, Congenital, Medicinal and Biomolecular Chemistry, Organic Chemistry, Pharmacology and Pharmaceutical Sciences, Medicinal & Biomolecular Chemistry
وصف الملف: application/pdf
URL الوصول: https://escholarship.org/uc/item/2xp6h9cz
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13Academic Journal
المصدر: Clinics. January 2014 69(2)
مصطلحات موضوعية: Genistein, Cystic Fibrosis Transmembrane Regulator, Oestrogen Receptor
وصف الملف: text/html
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14Academic Journal
المصدر: Clinics, Vol 69, Iss 2, Pp 111-119 (2014)
مصطلحات موضوعية: Genistein, Cystic Fibrosis Transmembrane Regulator, Oestrogen Receptor, Medicine (General), R5-920
وصف الملف: electronic resource
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15Academic Journal
المؤلفون: Khukhlina, O. S., Ursul, O. O., Voyevidka, O. S., Kaniovska, L. V., Gaydichuk, V. S., Andrusiak, A. V.
المصدر: Клінічна та експериментальна патологія; Том 16 № 2 (2017) ; Клиническая и экспериментальная патология; Том 16 № 2 (2017) ; Clinical & experimental pathology; Vol. 16 No. 2 (2017) ; 1727-4338
مصطلحات موضوعية: chronic obstructive pulmonary disease, chronic pancreatitis, cystic fibrosis, cystic fibrosis transmembrane regulator protein, smoking, хроническое обструктивное заболевание легких, хронический панкреатит, муковисцидоз, белок трансмембранного регулятора проводимости муковисцидоза, курения, хронічне обструктивне захворювання легень, хронічний панкреатит, муковісцидоз, білок трансмембранного регулятора провідності муковісцидозу, куріння
وصف الملف: application/pdf
Relation: http://cep.bsmu.edu.ua/article/view/1727-4338.XVI.2.60.2017.22/108632; http://cep.bsmu.edu.ua/article/view/1727-4338.XVI.2.60.2017.22
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16
المؤلفون: Melinda A. Engevik, Amy C. Engevik
المصدر: Computational and Structural Biotechnology Journal
Computational and Structural Biotechnology Journal, Vol 19, Iss, Pp 134-144 (2021)مصطلحات موضوعية: Gastrointestinal, SGLT1, sodium glucose co-transporter 1, Biophysics, Review Article, Gut flora, Biochemistry, digestive system, CFTR, cystic fibrosis transmembrane regulator, NHE2, sodium-hydrogen exchanger isoform 2, 03 medical and health sciences, 0302 clinical medicine, Immune system, Structural Biology, Genetics, Microbiome, CFTR, OTUs, operational taxonomic units, Ion transporter, 030304 developmental biology, Ion transport, 0303 health sciences, Gastrointestinal tract, biology, Tight junction, Host (biology), GLUT2, glucose transporter 2, Microbiota, NHE2, NHE3, sodium-hydrogen exchanger isoform 3, NHE3, ENaC, epithelial Na+ channel, biology.organism_classification, GI, gastrointestinal, NKCC1, Na+-K+-2Cl− co-transporter, DRA, Computer Science Applications, Cell biology, DRA, down-regulated in adenoma, 030220 oncology & carcinogenesis, Intestinal Microbiome, ClC, chloride channel, TP248.13-248.65, Biotechnology, GLUT2
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17
المؤلفون: Uhl, Franziska E., Vanherle, Lotte, Matthes, Frank, Meissner, Anja
المصدر: International Journal of Molecular Sciences, Vol 23, Iss 866, p 866 (2022)
International Journal of Molecular Sciencesمصطلحات موضوعية: Cystic Fibrosis, QH301-705.5, Cystic Fibrosis Transmembrane Conductance Regulator, Gene Expression, heart failure, lung, Mice, cystic fibrosis transmembrane regulator, Sphingosine, T-Lymphocyte Subsets, Animals, ddc:610, Biology (General), QD1-999, Communication, Pneumonia, respiratory system, respiratory tract diseases, Disease Models, Animal, Chemistry, Organ Specificity, inflammation, sphingosine-1-phosphate, lipids (amino acids, peptides, and proteins), Disease Susceptibility, Lysophospholipids, Biomarkers, Signal Transduction
وصف الملف: application/pdf
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18Academic Journal
المؤلفون: Ramin Radpour, Iman Salahshourifar, Hamid Gourabi, Mohamad AN Sadighi Gilani, Ahmad Vosough Dizaj
المصدر: International Journal of Fertility and Sterility, Vol 1, Iss 1, Pp 1-10 (2007)
مصطلحات موضوعية: cystic fibrosis transmembrane regulator (cftr), congenital bilateral absence of vas deferens (cbavd), Medicine (General), R5-920
وصف الملف: electronic resource
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19
المؤلفون: Franziska E. Uhl, Lotte Vanherle, Anja Meissner
مصطلحات موضوعية: Immunology, Applied Immunology (incl. Antibody Engineering, Xenotransplantation and T-cell Therapies), Autoimmunity, Cellular Immunology, Humoural Immunology and Immunochemistry, Immunogenetics (incl. Genetic Immunology), Innate Immunity, Transplantation Immunology, Tumour Immunology, Immunology not elsewhere classified, Genetic Immunology, Animal Immunology, Veterinary Immunology, lung, heart failure, cystic fibrosis transmembrane regulator, inflammation, macrophages
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20
المؤلفون: Asha Dube, Mubashar Iqbal, Mahan Salehi, Amer Al-Joudeh, F P Edenborough
المصدر: Respiratory Medicine Case Reports
Respiratory Medicine Case Reports, Vol 34, Iss, Pp 101553-(2021)مصطلحات موضوعية: Pulmonary and Respiratory Medicine, Drug, Hepatic necrosis, medicine.medical_specialty, Side effect, EBV, Epstein-Barr Virus, media_common.quotation_subject, Severe transaminitis, Case Report, Liver injury, Adverse effect, Cystic fibrosis, Gastroenterology, CF, Cystic Fibrosis, Ivacaftor, Diseases of the respiratory system, Quality of life, Internal medicine, ALT, Alanine Transaminase, medicine, CMV, Cytomegalovirus, media_common, CFTRm, Transmembrane Regulator Protein Modulator, RC705-779, business.industry, VZV, Varicella-Zoster Virus, FEV1, Forced Expiratory Volume, medicine.disease, CFTR modulators, ALP, Alkaline Phosphatase, Transaminitis, AST, Aspartate Transaminase, business, GGT, Gamma-Glutamyl Transferase, medicine.drug