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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 | Saccharomyces cerevisiae CNCM I-3856 in irritable bowel syndrome: An individual subject meta-analysis显示文摘AIM To confirm previous conclusions on Saccharomyces cerevisiae(S. cerevisiae) CNCM I-3856 for irritable bowel syndrome(IBS) management.METHODS An individual patient data meta-analysis was performed on two randomized clinical trials studying the effect of S. cerevisiae CNCM I-3856 supplementation on gastrointestinal(GI) symptoms in IBS subjects. A total of 579 IBS subjects were included. Outcomes were the daily Likert scale scores of abdominal pain/discomfort and bloating [area under the curve(AUC) and weekly means], responder status, and bowel movements(stool frequency and consistency). Statistical analyses were conducted in Intent to Treat(ITT) population, IBS-C subjects and IBS-C subjects with an abdominal pain/discomfort score higher than or equal to 2 at baseline('IBS-C ≥ 2 subpopulation').RESULTS S. cerevisiae CNCM I-3856 significantly improved abdominal pain/discomfort and bloating during the second month of supplementation [AUC(W5-W8)]with improvement up to the minimal clinically relevant threshold of 10%: a 12.3% reduction of abdominal pain/discomfort in the ITT population compared to the Placebo group(P = 0.0134) has been observed. In the IBS-C ≥ 2 subpopulation, there were a 13.1% reduction of abdominal pain/discomfort and a 14.9% reduction of bloating compared to the Placebo group(P = 0.0194 and P = 0.0145, respectively). GI symptoms significantly decreased during supplementation but no statistical differences were reported between groups at the end of the supplementation period. Responder status was defined as a subject who experienced a decrease of 1 arbitrary unit(a.u.) or 50% of the abdominal discomfort score from baseline for at least 2 wk out of the last 4 wk of the study. A significant difference between groups was reported in the ITT population, when considering the first definition: subjects in the Active group had 1.510 higher odds to be a responder(reduction of 1 a.u. of abdominal pain/discomfort) compared with subjects in the Placebo group(P = 0.0240). At the end of supplementation period, stool consistency in the Active group of the ITT population was significantly improved and classified as 'normal' compared to Placebo(respectively 3.13 ± 1.197 a.u. vs 2.58 ± 1.020 a.u., P = 0.0003). Similar results were seen in the IBS-C ≥ 2 subpopulation(Active group: 3.14 ± 1.219 a.u. vs Placebo group: 2.59 ± 1.017 a.u., P = 0.0009).CONCLUSION This meta-analysis supports previous data linking S. cerevisiae I-3856 and improvement of GI symptoms, in IBS overall population and in the IBS-C and IBS-C ≥ 2 subpopulations. | Amélie Cayzeele-Decherf Fanny Pélerin Sébastien Leuillet Benoit Douillard Béatrice Housez Murielle Cazaubiel Gunnard K Jacobson Peter Jüsten Pierre Desreumaux | 2017 | World Journal of Gastroenterology2017，23，2： | 2 |
| 3 | Wnt signalling regulates adult hippo - campal neurogenesis显示文摘 | Lie DC Colamarino SA Song H J | 2005 | Nature2005，437，7063： | 1 |
| 4 | Annual and seasonal variations of the sea surface heat fluxes in the East Asian marginal seas显示文摘 | Na J Seo J Lie H J | 1999 | J Oceanogr1999，55，： | 1 |
| 5 | Estimating parameters of intensity function and maintenance effectfor repairable unit显示文摘 | Shin I Lim T J Lie C H | 1996 | Reliability Engineering and System Safety1996，54，1： | 1 |
| 6 | A Practical Simple Geometry and Gain/Phase Calibration Technique for An- tenna Array Processing显示文摘 | Ng B P Lie J P Er M H Feng A | 2009 | IEEE Transactions on Anten- nas and Propagation2009，57，7： | 1 |
| 7 | Robust adaptive beamforming based on covariance matrix reconstruction for look direction mismatch显示文摘 | Mallipeddi R Lie J P Suganthan P N | 2011 | Progress in Electromagnetics Research Letters2011，25，： | 1 |
| 8 | Human papillomavirus infection as a risk factor for squamous-cell carcinoma of the head and neck显示文摘 | Mork J Lie A K Glattre E | | 0，，15： | 1 |
| 9 | Fine grid tidal modeling of the Yellow and East China Seas显示文摘 | KANG S K LEE S R LIE H J | 1998 | Continental Shelf Research1998，18，： | 1 |
| 10 | Pharmaco-kinetics of deferiprone in patients with 茁-thalassaemia显示文摘 | LIE M G L TOTSAPOL J PIYADA J | 2011 | Clin Phamracokinet2011，50，1： | 1 |
| 11 | long-term prog-nosis after operation for adhesive small bowel obstruction显示文摘 | FEVANG BT FEVANG J LIE | 2004 | Am Surg2004，240，2： | 1 |
| 12 | Wnt signal- ling regulates adult hippocampal neurogenesis 显示文摘 | Lie D C Colamarino S A Song H J | 2005 | Na- ture2005，437，7063： | 1 |
| 13 | Anterior cervical discectomy with fusion in patients with cervical disc degeneration: a prospective outcome study of 258 patients ( 181 fused with autologous bone graft and 77 fused with a PEEK cage) 显示文摘 | Lied B Roenning PA Sundseth J | 2010 | BMC Surg2010，0，1： | 1 |
| 14 | UGT1A6genotype-related pharmacokinetics of deferiprone ( L1) inhealthy volunteers显示文摘 | LIE M G L TOTSAPOL J JEERANUT T | 2008 | Br J Clin Pharmacol2008，65，6： | 1 |
| 15 | Least squares support vector machine classifiers显示文摘 | Suykens J Vandewa Lie J | 1999 | Neural Processing Letters1999，9，3： | 1 |
| 16 | System voltage sag performance estimation显示文摘 | Wang J Chen S Lie T T | 2005 | IEEE Trans on Power Delivery2005，20，2： | 1 |
| 17 | Method to calculate the fire resistance of reinforced concrete columns with rectangular cross section显示文摘 | Lie T T Irwin R J | 1993 | ACI Structural Journal1993，90，1： | 1 |
| 18 | Direct measurement of charge current by employing a mesh Electrode in the laser plasma induced by Nd, YAG laser (I) 显示文摘 | Pardede M Kumiawan H Lie T J | 2002 | Appl Spectrosc2002，56，8： | 1 |
| 19 | Elevation of plasma phospholipid transfer protein in transgenic mice increases VLDL secretion显示文摘 | LIE J DE CROM R VAN GENT T | 2002 | J Lipid Res2002，43，11： | 1 |
| 20 | Fast computation of multiphase flow in porous media by implicit discontinuous Galerkin schemes with optimal ordering of elements显示文摘 | NATVIG J LIE K | 2008 | Journal of Computational Physics2008，227，10： | 1 |
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