Phenotypic characterization and comparison of Phe508del and cystic fibrosis transmembrane conductance regulator (CFTR) knockout rat models of cystic fibrosis generated by CRISPR/Cas9 gene editing.
暂无分享,去创建一个
M. Donnelley | J. Finnie | R. Boucher | D. Parsons | B. Boog | R. Gilmore | N. Rout-Pitt | J. Delhove | P. Cmielewski | A. McCarron | Chantelle McIntyre | W. O’Neal | H. Chan | N. Reyne | Fiona Craig | J. E. Schjenken | E. Knight
[1] P. Negulescu,et al. In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis , 2019, Science Translational Medicine.
[2] R. Croll,et al. Animal Models in the Pathophysiology of Cystic Fibrosis , 2019, Front. Pharmacol..
[3] A. Harris,et al. A sheep model of cystic fibrosis generated by CRISPR/Cas9 disruption of the CFTR gene. , 2018, JCI insight.
[4] Martin Donnelley,et al. Airway disease phenotypes in animal models of cystic fibrosis , 2018, Respiratory Research.
[5] Guillermo J Tearney,et al. Development of an airway mucus defect in the cystic fibrosis rat. , 2018, JCI insight.
[6] Qili He,et al. Sex-specific reference intervals of hematologic and biochemical analytes in Sprague-Dawley rats using the nonparametric rank percentile method , 2017, PloS one.
[7] D. Meyerholz,et al. Lentiviral-mediated phenotypic correction of cystic fibrosis pigs. , 2016, JCI insight.
[8] D. Schaffer,et al. CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes. , 2016, JCI insight.
[9] D. Meyerholz. Lessons learned from the cystic fibrosis pig. , 2016, Theriogenology.
[10] N. McElvaney,et al. Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences , 2016, BioMed research international.
[11] D. Meyerholz,et al. Pancreatic pathophysiology in cystic fibrosis , 2016, The Journal of pathology.
[12] D. Meyerholz,et al. Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology. , 2015, American journal of physiology. Gastrointestinal and liver physiology.
[13] J. Olsen,et al. Ferret and pig models of cystic fibrosis: prospects and promise for gene therapy. , 2014, Human gene therapy. Clinical development.
[14] S. Matalon,et al. CFTR and lung homeostasis. , 2014, American journal of physiology. Lung cellular and molecular physiology.
[15] M. Donnelley,et al. Long‐term therapeutic and reporter gene expression in lentiviral vector treated cystic fibrosis mice , 2014, The journal of gene medicine.
[16] D. Sheppard,et al. Understanding how cystic fibrosis mutations disrupt CFTR function: from single molecules to animal models. , 2014, The international journal of biochemistry & cell biology.
[17] Samuel I. Miller,et al. Gastrointestinal pathology in juvenile and adult CFTR-knockout ferrets. , 2014, The American journal of pathology.
[18] K. Chu,et al. Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats , 2014, PloS one.
[19] S. Randell,et al. Loss of Cftr function exacerbates the phenotype of Na(+) hyperabsorption in murine airways. , 2013, American journal of physiology. Lung cellular and molecular physiology.
[20] J. Chen,et al. Regulation of male fertility by CFTR and implications in male infertility. , 2012, Human reproduction update.
[21] M. Welsh,et al. Reduced Airway Surface pH Impairs Bacterial Killing in the Porcine Cystic Fibrosis Lung , 2012, Nature.
[22] M. Drumm,et al. Variation in MSRA Modifies Risk of Neonatal Intestinal Obstruction in Cystic Fibrosis , 2012, PLoS genetics.
[23] L. Touqui,et al. Mouse models of cystic fibrosis: phenotypic analysis and research applications. , 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
[24] M. Conese,et al. Pharmacological therapy for cystic fibrosis: from bench to bedside. , 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
[25] S. Richter,et al. The ΔF508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis–Like Disease in Pigs , 2011, Science Translational Medicine.
[26] David K Meyerholz,et al. Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. , 2010, The Journal of clinical investigation.
[27] Jason C. Young,et al. Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane , 2010, Science.
[28] S. Richter,et al. Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth , 2010, Science Translational Medicine.
[29] D. Meyerholz,et al. Pathology of gastrointestinal organs in a porcine model of cystic fibrosis. , 2010, The American journal of pathology.
[30] S. Gallati,et al. The CFTR frameshift mutation 3905insT and its effect at transcript and protein level , 2010, European Journal of Human Genetics.
[31] M. Corey,et al. Modifier gene study of meconium ileus in cystic fibrosis: statistical considerations and gene mapping results , 2009, Human Genetics.
[32] S. Stepaniants,et al. Selection of housekeeping genes for real-time PCR in atopic human bronchial epithelial cells , 2008, European Respiratory Journal.
[33] M. Welsh,et al. Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer. , 2008, The Journal of clinical investigation.
[34] M. Welsh,et al. Processing and function of CFTR-ΔF508 are species-dependent , 2007, Proceedings of the National Academy of Sciences.
[35] A. Chakravarti,et al. Relative contribution of genetic and nongenetic modifiers to intestinal obstruction in cystic fibrosis. , 2006, Gastroenterology.
[36] N. Joo,et al. Hyposecretion, Not Hyperabsorption, Is the Basic Defect of Cystic Fibrosis Airway Glands*♦ , 2006, Journal of Biological Chemistry.
[37] M. Amaral. Processing of CFTR: Traversing the cellular maze—How much CFTR needs to go through to avoid cystic fibrosis? , 2005, Pediatric pulmonology.
[38] A. Verkman,et al. Submucosal gland dysfunction as a primary defect in cystic fibrosis , 2005, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.
[39] C. Ackerley,et al. Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model. , 2004, The American journal of pathology.
[40] J. Widdicombe,et al. Distribution of tracheal and laryngeal mucous glands in some rodents and the rabbit , 2001, Journal of anatomy.
[41] L. Tsui,et al. Cystic fibrosis gene mutations and infertile men with primary testicular failure , 2000 .
[42] M. Wilschanski,et al. Pathology of pancreatic and intestinal disorders in cystic fibrosis , 1998, Journal of the Royal Society of Medicine.
[43] B. Grubb,et al. Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis. , 1997, The American journal of physiology.
[44] J. T. Wright,et al. Abnormal Enamel Development in a Cystic Fibrosis Transgenic Mouse Model , 1996, Journal of dental research.
[45] C. Ober,et al. Cystic fibrosis mutation screening in healthy men with reduced sperm quality. , 1996, Human reproduction.
[46] B. Abella,et al. Generation and characterization of a ΔF508 cystic fibrosis mouse model , 1995, Nature Genetics.
[47] James M. Wilson,et al. Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans , 1994, Nature.
[48] James M. Wilson,et al. Expression of the cystic fibrosis gene in adult human lung. , 1994, The Journal of clinical investigation.
[49] A. Miller,et al. Non–invasive liposome–mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice , 1993 .
[50] M. Evans,et al. Production of a severe cystic fibrosis mutation in mice by gene targeting , 1993, Nature Genetics.
[51] V. Ferrans,et al. Expression of the cystic fibrosis transmembrane conductance regulator gene in the respiratory tract of normal individuals and individuals with cystic fibrosis. , 1991, Proceedings of the National Academy of Sciences of the United States of America.
[52] J. Marshall,et al. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis , 1990, Cell.
[53] L. Tsui,et al. Erratum: Identification of the Cystic Fibrosis Gene: Genetic Analysis , 1989, Science.
[54] J. Opitz,et al. Pathological confirmation of cystic fibrosis in the fetus following prenatal diagnosis. , 1987, American journal of medical genetics.
[55] J. T. Young. Histopathologic examination of the rat nasal cavity. , 1981, Fundamental and applied toxicology : official journal of the Society of Toxicology.
[56] J. Maloney,et al. New features in the development of the submucosal gland of the respiratory tract. , 1978, Journal of anatomy.
[57] H. Shwachman,et al. Reproductive failure in males with cystic fibrosis. , 1968, The New England journal of medicine.
[58] E. Porta,et al. ULTRASTRUCTURAL CHANGES OF THE PANCREAS AND LIVER IN CYSTIC FIBROSIS. , 1964, American journal of clinical pathology.
[59] D. Andersen. CYSTIC FIBROSIS OF THE PANCREAS AND ITS RELATION TO CELIAC DISEASE: A CLINICAL AND PATHOLOGIC STUDY , 1938 .
[60] N. Joo,et al. Submucosal glands and airway defense. , 2004, Proceedings of the American Thoracic Society.
[61] A. Verkman,et al. Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease. , 2003, American journal of physiology. Cell physiology.
[62] R. Boucher,et al. Pathophysiology of gene-targeted mouse models for cystic fibrosis. , 1999, Physiological reviews.
[63] R. Kopito,et al. Biosynthesis and degradation of CFTR. , 1999, Physiological reviews.
[64] Esterly,et al. Pathology of cystic fibrosis review of the literature and comparison with 146 autopsied cases. , 1975, Perspectives in pediatric pathology.