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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 | Evaluation of fibroblast at- tachment in root conditioning with Er, Cr:YSGG laser versus ED- TA:A SEM study 显示文摘 | Fekrazad R Lotfi G Harandi M | 2015 | Microsc Res Tech2015，78，4： | 1 |
| 3 | Interface model applied to fracture of masonry structures 显示文摘 | Lotfi H R Shing B | 1994 | Journal of Structural Engineering1994，120，1： | 1 |
| 4 | Evolutionary origins,diversification,and biogeography of liver flukes(Digenea,Fasciolidae)显示文摘 | LOTFY W M BRANT S V DEJONG R J | 2008 | Am J Trop Med Hyg2008，79，2： | 1 |
| 5 | Degradation of chitosan and starch by360-KHZ ultrasound显示文摘 | Czecowska-Biskup R Rokita B Lotfy S | 2005 | Carbohydrate polymers2005，60，2： | 1 |
| 6 | An algorithmic method to extend TOPSIS for decision-making problems with interval data显示文摘 | G R JAHANSHAHLOO F H LOTFI M IZADIKHAH | | 0，，02： | 1 |
| 7 | Extension of the TOPSIS method for decision making problems with fuzzy data显示文摘 | Jahanshahloo G R Lotfi F H Izadikhah M | 2006 | Journal of Applied Mathematics2006，181，2： | 1 |
| 8 | HMGB1 is overexpressed in tumor cells and promotes activity of regulatory T cells in patients with head and neck cancer显示文摘 | Wild CA Brandau S Lotfi R | 2012 | Oral Oncol2012，48，5： | 1 |
| 9 | An algorithmic method to extend TOPSIS for decision-mak- ing problems with interval data显示文摘 | JAHANSHAHLOO G R LOTFI F H IZADIKHAH M | 2006 | Applied Mathematics and Computation2006，175，2： | 1 |
| 10 | Interface model applied to fracture of masonry structures显示文摘 | LOTFI H R SHING P B | 1994 | Journal of Structural Engineering ASCE1994，120，1： | 1 |
| 11 | An algorithmic method to extend TOPSIS for decision-making problems with interval data显示文摘 | JAHANSHAHLOO G R LOTFI F H IZADIKHAH M | 2006 | Applied Mathematics and Computation2006，175，2： | 1 |
| 12 | Extension of theTOPSIS method for decision-making problems with fuzzy data显示文摘 | Jahanshahloo G R Hosseinzadeh Lotfi F Izadikhah M | 2006 | App liedMathematics and Computation2006，181，： | 1 |
| 13 | Analysis of Power Consumption and Linearity in Capacitor Digital-to-analog Converters Used in Successive Approximation ADCs 显示文摘 | Saberi M Lotfi R Mafinezhad K | 2011 | IEEE Transactions on Circuits and Systems:Regular Papers2011，58，8： | 1 |
| 14 | Imprecise shannon’s entropy and multi attribute decision making 显示文摘 | Lotfi H Fallahnejad R | 2010 | Entropy2010，12，1： | 1 |
| 15 | Interface model applied to fracture of masonry structures 显示文摘 | LOTFI H R SHING P B | 1994 | Journal of Structur- al Engineering1994，120，1： | 1 |
| 16 | An algorithmic method to extend TOPSIS for decision-making problems with interval data显示文摘 | Jahanshahloo G R Hosseinzadeh Lotfi F Izadikhah M | 2006 | Applied Mathematics and Computation2006，175，： | 1 |
| 17 | A study on comparative outcomes of percutaneous nephrolithotomy in prone, supine, and flank positions显示文摘 | Karami H Mohammadi R Lotfi B | 2013 | World J Urol2013，31，： | 1 |
| 18 | Analysis and design of a low-voltage low-power double-tTail comparator 显示文摘 | BABAYAN-MASHHADI S LOTFI R | 2014 | IEEE Trans VLSI Syst2014，22，2： | 1 |
| 19 | An algorithmic method to extend TOPSIS for decision-making problems with interval data显示文摘 | Jahanshahloo G R Hosseinzadeh Lotfi F Izadikhah M | 2006 | Applied Mathematics and Computation2006，175，： | 1 |
| 20 | Selecting symmetric weights as a secondary goal in DEA cross-efficiency evaluation 显示文摘 | Jahanshahloo G R Hosseinzadeh Lotfi F Jafari Y | 2011 | Applied Mathematical Modelling2011，35，： | 1 |
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