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| 1 | Characterization of two rat models of cystic fibrosis—KO and F508del CFTR—Generated by Crispr-Cas9显示文摘Background: Genetically engineered animals are essential for gaining a proper understanding of the disease mechanisms of cystic fibrosis(CF). The rat is a relevant laboratory model for CF because of its zootechnical capacity, size, and airway characteristics, including the presence of submucosal glands.Methods: We describe the generation of a CF rat model(F508 del) homozygous for the p.Phe508 del mutation in the transmembrane conductance regulator(Cftr) gene. This model was compared to new Cftr-/-rats(CFTR KO). Target organs in CF were examined by histological staining of tissue sections and tooth enamel was quantified by micro-computed tomography. The activity of CFTR was evaluated by nasal potential difference(NPD) and short-circuit current measurements. The effect of VX-809 and VX-770 was analyzed on nasal epithelial primary cell cultures from F508 del rats.Results: Both newborn F508 del and Knock out(KO) animals developed intestinal obstruction that could be partly compensated by special diet combined with an osmotic laxative. The two rat models exhibited CF phenotypic anomalies such as vas deferens agenesis and tooth enamel defects. Histology of the intestine, pancreas, liver, and lungs was normal. Absence of CFTR function in KO rats was confirmed ex vivo by short-circuit current measurements on colon mucosae and in vivo by NPD, whereas residual CFTR activity was observed in F508 del rats. Exposure of F508 del CFTR nasal primary cultures to a combination of VX-809 and VX-770 improved CFTR-mediated Cl-transport.Conclusions: The F508 del rats reproduce the phenotypes observed in CFTR KO animals and represent a novel resource to advance the development of CF therapeutics. | Elise Dreano Marc Bacchetta Juliette Simonin Louise Galmiche Claire Usal Lotfi Slimani Jérémy Sadoine Laurent Tesson Ignacio Anegon Jean-Paul Concordet Aurélie Hatton Lucile Vignaud Danielle Tondelier Isabelle Sermet-Gaudelus Marc Chanson Charles-Henry Cottart | 2019 | Animal Models and Experimental Medicine2019，2，4： | 3 |
| 2 | European Prospective Inves?tigation into Cancer and Nutrition (EPIC) : study populations and data collection显示文摘 | Riboli E Hunt KJ Slimani N | 2002 | Public Health Nutr2002，5，6： | 1 |
| 3 | Nondifferentiable Multiobjective Programming under Generalized df-Invexity显示文摘 | Slimani H Radjef M S | 2010 | EuropeanJournal of Operational Research2010，，： | 1 |
| 4 | Dietary fibre in food and protection against colorectal cancer in the European Prospective Investigation into Cancer and Nutrition (EPIC): an observational study显示文摘 | Sheila A Bingham Nicholas E Day Robert Luben Pietro Ferrari Nadia Slimani Teresa Norat Fran?oise Clavel-Chapelon Emmanuelle Kesse Alexandra Nieters Heiner Boeing Anne Tj?nneland Kim Overvad Carmen Martinez Miren Dorronsoro Carlos A Gonzalez Timothy J Key | 2003 | The Lancet2003，，9368： | 1 |
| 5 | Standardisation of an European end-user nutrient database for nutritional epidemiology:what can we learn from the EPIC Nutrient Database(ENDB) Project显示文摘 | Slimani N Deharveng G Unwin I | 2007 | Trends Food Sci Tech2007，18，8： | 1 |
| 6 | Analysis and implementation of a real-time predictive current controller for permanent-magnet synchronous servo drives显示文摘 | Huy L H Slimani K Viarouge P | 1994 | IEEE Transactions on Industrial Electronics1994，41，1： | 1 |
| 7 | A poly(lactic-co-glycolic acid)knitted scaffold for tendon tissue engineering:an in vitro and in vivo study显示文摘 | Vaquette C Slimani S Kahn CJ | | 0，，13： | 1 |
| 8 | Nondifferentiable Muhiobjective Programming under Generalized d l Invexity显示文摘 | Slimani H Radjef M S | 2010 | European Journal of Operational Research2010，202，1： | 1 |
| 9 | An evaluation of the relative efficacy of an open airway,an oxygen reservoir and continuous positive airway pressure 5 cmH2O on the non-ventilated lung显示文摘 | Slimani J Russell WJ Jurisevic C | 2004 | Anaesth Intensive Care2004，32，6： | 1 |
| 10 | Hsp70-2 gene polymorphism:susceptibility implication in Tunisian patients with coronary artery disease显示文摘 | Hrira MY Chkioua L Slimani A | 2012 | Diagn Pathol2012，7，1： | 1 |
| 11 | Pressure transient analysis ofpartially penetrating wells in a naturally fractured reservoir显示文摘 | Slimani K Tiab D Moncada K | 2006 | SPE2006，，： | 1 |
| 12 | The French Haemovigilance System: organization and results for 2003显示文摘 | Rebibo D Hauser L Slimani A | 2004 | Transfus Apher Sci2004，31，2： | 1 |
| 13 | The French Haemovigilance System: organization and results for 2003显示文摘 | Rebibo D Hauser L Slimani A | 2004 | Transfus Apher Sci2004，31，2： | 1 |
| 14 | Extension of the one-shot semijoin strategy to minimize data transmission cost in distributed query processing显示文摘 | Faiza Najjar Yahya Slimani | 1999 | Information Science1999，114，： | 1 |
| 15 | Analysis and implementation of a real'time predictive currentcontroller for permanent-magnet synchronous servo drives显示文摘 | Huy L H Slimani K Viarouge P | 1994 | IEEE Transactions on Industrial Electronics1994，41，1： | 1 |
| 16 | European Prospective Investigation into Cancer and Nutrition (EPIC) calibration study: rationale, design and population characteristics 显示文摘 | SLIMANI N KAAKS R FERRARI P | 2002 | Public Health Nutrition2002，6，5： | 1 |
| 17 | Analysis and implementation of a real-time predictive current controller for permanent-magnet synchronous servo drives显示文摘 | HOANG L H SLIMANI K VICARAGE P | 2013 | IEEE Transactions on Industrial Electronics2013，，： | 1 |
| 18 | Prevalence of rheumatoid arthritis in an urban population of Algeria:a prospective study显示文摘 | Slimani S Ladjouze-Rezig A | 2014 | Rheumatology2014，53，3： | 1 |
| 19 | Meat,fish and colorectal cancer risk:the European prospective investigation into cancer and nutrition显示文摘 | Norat T Bingham S Ferrari P Slimani N Jena M Mazur M | | 0，，： | 1 |
| 20 | Ultra- sound diagnosis of ectopic pregnancy : prospective study of 200 cases显示文摘 | MATHLOUTHI N SLIMANI O FATNASSI A | 2013 | Tunis Med2013，91，4： | 1 |
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