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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 | Lung cancer in radon-exposed miners and estimation of risk from indoor exposure显示文摘 | DARBY S C ELISE W HOWE G R | 1995 | Journal of the Na-tional Cancer Institute1995，87，5： | 1 |
| 3 | Prevalence and risk factors for adenomas in the ileal pouch and the afferent loop after restorative proctocolectomy for patients with familial adenomatous polyposis显示文摘 | Elise Pommaret Ariane Vienne Jérémie H. Lefevre Philippe Sogni Christian Florent Beno?t Desaint Yann Parc | 2013 | Surgical Endoscopy2013，，10： | 1 |
| 4 | Purification and properties of the extracellular lipase, LipA, of Acinetobacter sp RAG - 1 显示文摘 | ERICK A SNELLMAN ELISE R | 2002 | Eur J Biochem2002，269，： | 1 |
| 5 | Zinc supplementation provides behavioral resiliency in a rat model of traumatic brain injury显示文摘 | Elise C Deborah R Anugs G | 2011 | Physiol Behav2011，104，5： | 1 |
| 6 | Synthesis, characterization and properties of poly(ethylene-terephthalate)/poly (1,4-buty-lene succinate) block copolymers显示文摘 | Darwin P R Kint Abdelilah Alla Elise Deloret | 2003 | Polymer2003，44，： | 1 |
| 7 | Synthesis of acrylic - polyurethane hybrid latexes by miniemulsion polymerization and their pressure - sensitive adhesive applications显示文摘 | Udagama R Elise D C Creton C | 2011 | Macromolecules2011，44，8： | 1 |
| 8 | Managing stakeholders--A Tourism Planning Model显示文摘 | SAUTTE R ELISE Trtlly BIRGIT Leisen | 1999 | Annals of Tourism Research1999，26，2： | 1 |
| 9 | Geophysical investigation using resistivity and GPR methods:a case study of a lubricant oil waste disposal area in the city of Ribeirao Preto,Sāo Paulo,Brazil显示文摘 | Lago A L Elis V R Borges W R | | 0，，02： | 1 |
| 10 | Isolation of RNA and DNA from rice seedling mitochondria 显示文摘 | MICHAEL M R ELISE D CHAZ A | 1988 | Rice Genet Newsl1988，5，： | 1 |
| 11 | Molecular genetics of biosurfactant production显示文摘 | Elise R S | 1998 | Current Opinion in Biotechnology1998，9，： | 1 |
| 12 | Increased eosinophil-lineage committed progenitors in the lung of allergen-chal-lenged mice显示文摘 | Southam DS Widmer N Elis R | | 0，，01： | 1 |
| 13 | The sensitivity of neutro- phil CD64 expression as a biomarker of bacterial infection is low in critically ill patients 显示文摘 | Antoine G Mikael R Elise S | 2012 | Intensive Care Med2012，38，12： | 1 |
| 14 | Leapfrogging in international competition: a theory of cycles in national technology leadership 显示文摘 | ELISE S PAUL R K DANIEL T | 2004 | The American Economic Review2004，83，5： | 1 |
| 15 | Leapfrogging in international competition : A theory of cycles in national techonlogical leadership显示文摘 | Brezis Elise S Krugman Paul R Tsiddon Daniel | 1993 | American Economic Review1993，83，12： | 1 |
| 16 | Clinical and biologic behavior of bone metastases from differentianted thyroid carcinoma显示文摘 | Marcocci C Pacini F Elise R | 1989 | Surgery1989，106，6： | 1 |
| 17 | Human intoxication by microcystins during renal dialysis treatment in Caruaru—Brazil显示文摘 | Sandra M.F.O Azevedo Wayne W Carmichael Elise M Jochimsen Kenneth L Rinehart Sharon Lau Glen R Shaw Geoff K Eaglesham | 2002 | Toxicology2002，，： | 1 |
| 18 | Technology and the Life Cycle of Cities显示文摘 | Elise S B Paul R K | 1997 | Journal of Economic Growth1997，2，： | 1 |
| 19 | Early detection of ketoprofen-induced acute kidney injury in sheep as be- termined by evaluation of urinary enzyme activities显示文摘 | Maria R Elise MK Minna M | 2010 | American Journal of Veterinary Research2010，71，10： | 1 |
| 20 | Isolation of RNA and DNA from rice seedling mitochondria显示文摘 | MICHAEL M R ELISE D CHAZ A | 1988 | Rice Genetics Newsletters1988，5，： | 1 |
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