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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 | Improved control of postharvest decay of pears by the combination of Candida sake (CPA 1) and ammonium molybdate 显示文摘 | Nunes C Usall J Teixido N | 2002 | Phytopathology2002，92，： | 1 |
| 3 | Efficacy of Neutral Electrolyzed Water (NEW) for Reducing Microbial Contamination on Minimally-processed Vegetables显示文摘 | ABADIAS M USALL J OLIVEIRA M | 2008 | Journal of Food Microbiology2008，123，： | 1 |
| 4 | Efficacy of neutral electrolyzed water (NEW) for reducing microbial contamination on minimally-processed vegetables显示文摘 | ABADIAS M USALL J OLIVEIRA M | 2008 | International Journal of Food Microbiology2008，123，1： | 1 |
| 5 | Improving ecological fitness and environmental stress tolerance of the biocontrol yeast Candida sake by manipulation of intraceUuar sugar alcohol and sugar content显示文摘 | TEIXIDO N VINAS I USALL J | 1998 | MycologicalResearch1998，102，11： | 1 |
| 6 | Control of blue mold of apple by preharvest application of Candida sake grown in media with different water activity显示文摘 | TEIXIDO N VINAS I USALL J | 1998 | Phytopathology1998，88，9： | 1 |
| 7 | Antagonistic effect of Pseudomonas graminis CPA-7 against foodbome pathogens in fresh-cut apples under simulated commercial conditions显示文摘 | Alegrc I Vinas I Usall J ct al | 2013 | Food microbiology2013，33，2： | 1 |
| 8 | Effect of protective agents,rehydration media and initial ceil concentration on viability of Pantoea agglomerans strain CPA - 2 subjected to freeze - drying 显示文摘 | Costa E Usall J Teixido N | 2000 | Journal of Applied Microbiology2000，89，： | 1 |
| 9 | Effect of water activity and temperature on competing abilities of common postharvest citrus fungi 显示文摘 | Pilar P Usall J | 2004 | Int J Food Microbiol2004，90，： | 1 |
| 10 | Suicide ideation across reproductive life cycle of women Results from a European epidemio-logical study显示文摘 | USALL J PINTO-MEZA A FERN/i NDEZ A | 2009 | J Affect Disord2009，116，12： | 1 |
| 11 | Survival of the postharvest biocontrol yeast Candida sake CPA-1 after dehydration by spray-drying 显示文摘 | ABADIAS M TEIXID N USALL J | 2005 | Bio Sci Technol2005，15，8： | 1 |
| 12 | Effect of biocontrol ageuts Candida sake and Pantoea agglomerans on Penicillium expansum growth and patulin accumulation in apples显示文摘 | MORALES H SANCHIS V USALL J | | 0，，1： | 1 |
| 13 | Renal macrophage activation and TH2 polarization precedes the development of nephrotic syndrome in Buffalo/Mna rats显示文摘 | Le Berre L Herve C Buzelin F Usal C Soulillou JP Dantal J | 2005 | Kidney Int2005，68，5： | 1 |
| 14 | Postharvest biological control by Pantoea agglomerans(CPA-2) on Golden Delicious apples显示文摘 | Nunes C Usall J Teixido N etal | 2002 | Journal of Applied Microbiology2002，92，2： | 1 |
| 15 | Gender differences in a sam pie of schizophrenic outpatients显示文摘 | Usall J Ochoa S Araya S | 2001 | Compr Psychiatry2001，42，4： | 1 |
| 16 | Effect of protective agents, rehydration media and initial cell concentration on viability of Pantoea agglomerans strain CPA-2 subjected to freeze- drying 显示文摘 | Costa E Usall J Teixido N | 2006 | Journal of Applied Microbiology2006，89，： | 1 |
| 17 | Nutritional enhance- ment of biocontrol of blue mold on apples 显示文摘 | JANISIEWICZ W J USALL J BORS B | 1992 | Phytopatholo- gy1992，82，： | 1 |
| 18 | Possible involvement of hydrogen peroxide in the development of resistance mechanisms in'Golden Delicious'apple fruit显示文摘 | Torres R Valentines M C Usall J | 2003 | Postharvest Biol Technol2003，27，： | 1 |
| 19 | Effect of protective agents, rehydration media and initial cell concentration on viability of Pantoea agglomerans strain CPA-2 subjected to freeze- drying显示文摘 | Costa E Usall J Teixido N | 2006 | Journal of Applied Microbiology2006，89，： | 1 |
| 20 | Preventive and curative activity of combined treatments of sodium car- bonates and Pantoea agglomerans CPA-2 to control postharvest green mold of citrus fruit显示文摘 | Usall J Smilanick J L Palou L | 2008 | Postharvest Bi- ology and Technology2008，50，： | 1 |
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