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	题名	作者	年代	出处	被引量
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	The diffusion- weighted imaging perfusion fraction f is a potential marker of sorafenib treatment in advanced hepatocellu- lar carcinoma: a pilot study显示文摘	Lewin M Fartoux L Vignaud A	2011	Eur Radiol2011，21，2：	1
3	The diffusion-weighted ima- ging peffusion fraction f is a potential marker of sorafenib treatment in advanced hepatocellular carcinoma:a pilot study显示文摘	Lewin M Fartoux L Vignaud A	2011	Eur Radiol2011，21，：	1
4	Separation and identification by gel filtration and high-performance liquid chromatography with UV or electrochemical detection of the disulphides produced from cysteine and glutathione oxidation 显示文摘	Vignaud C Rakotozafy L Falgieres A	2004	J Chromatogr A2004，1031，：	1
5	The role of cytokines in human lung fibrosis 显示文摘	Vaillant P Menard O Vignaud JM	1996	Manaldi Arch Chest Dis1996，51，2：	1
6	Imaging of the vestibule显示文摘	Vignaud J Marsot - Dupuch K Pharaboz C	1995	Otolaryngol Head Neck Surg1995，112，1：	1
7	The role of platelet-derived growth factor prodution by tumor-associated macrophages in tumor stroma formation in lung cancer 显示文摘	Vignaud JM Marie B Klein N	1994	Cancer Res1994，54，3：	1
8	In vitro and in vivo reversal of multidrug resistance by GF120918, an acridonecarboxamide derivative 显示文摘	Hyafil F Vergely C Du Vignaud P	1993	CancerRes1993，53，19：	1
9	Automation of metabolic stability studies in microsomes, cytosol and plasma using a 215 Gilson liquid handler显示文摘	Linget J M du Vignaud P	1999	J Pharm Biomed Anal1999，19，6：	1
10	Increased expression of the vascular endothelial growth factor is a pejorative prognosis marker in papillary thyroid carcinoma 显示文摘	KleinM Vignaud JM Hennequin V	2001	J Clin Endocrino Metab2001，86，2：	1
11	Vascular endothelial growth factor gene and protein : strong expression in thyroiditis and thyroid carcinoma显示文摘	Klein M Picard E Vignaud JM	1999	J Endocrinol1999，161，：	1
12	Liver cirrhosis:in- travoxel incoherent motion MR imaging-Pilot study显示文摘	Luciani A Vignaud A Cavet M	2008	Ra- diology2008，249，3：	1
13	Liver cirrhosis: intravoxel incoherent motion MR imaging--pilot study显示文摘	Luciani A Vignaud A Cavet M	2008	Radiology2008，249，3：	1
14	Vascular endothelial growth factor gene and protein: strong expression in thyroiditis and thyroid carcinoma显示文摘	Klein M Picard E Vignaud JM	1999	J Endocrinol1999，161，1：	1
15	显示文摘	Vsillat P Menard O Vignaud JM	1996	Monaldi Arch Chest Dis1996，51，2：	1
16	The diffusion-weighted imaging perfusion fraction a potential marker of sorafenib treatment in advanced epatocellular carcinoma: a pilot study 显示文摘	Lewin M Fartoux L Vignaud A	2011	Eur Radiol2011，21，2：	1
17	Liver cirrhosis:intravoxel incoherent motion MR imaging-pilot studY显示文摘	Luciani A Vignaud A Caret M	2008	Radiology2008，249，3：	1
18	Refeeding syndrome influences outcome of anorexia nervosa patients in intensive care unit:an observational study显示文摘	Vignaud M Constantin JM Ruivard M	2010	Crit Care2010，14，5：	1
19	Sustained peripheral arterial insufficiency durably impairs normal and regenerating skeletal muscle function显示文摘	Hourde C Vignaud A Beurdy I	2006	J Physiol Sci2006，56，5：	1
20	Liver cirrhosis: in- travoxel incoherent motion MR imaging-pilot study 显示文摘	Luciani A Vignaud A Cavet M	2008	Radiology2008，249，3：	1

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