Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs

1.Andersen DH. Pathology of cystic fibrosis. Ann NY Acad Sci. 1962;93:500–17.2.Oppenheimer EH, Esterly JR. Pathology of cystic fibrosis evaluate of the literature and comparability with 146 autopsied instances. Perspect Pediatr Pathol. 1975;2:241–78.three.Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352:1992–2001.four.Welsh MJ, Ramsey BW, Accurso F, Slicing GR. Cystic fibrosis. In: Scriver CR, Beaudet AL, Sly WS, Valle D, Childs B, Vogelstein B, editors. The metabolic and molecular foundation of inherited illness. New York, NY: McGraw-Hill; 2001. p. 5121–89. .5.VanDevanter DR, Kahle JS, O’Sullivan AK, Sikirica S, Hodgkins PS. Cystic fibrosis in younger youngsters: a evaluate of illness manifestation, development, and response to early therapy. J Cyst Fibros. 2015;15:147–57.6.Stoltz DA, Meyerholz DK, Welsh MJ. Origins of cystic fibrosis lung illness. N Engl J Med. 2015;372:351–62.7.Fischer AJ, Singh SB, Adam RJ, et al. Tracheomalacia is related to decrease FEV and Pseudomonas acquisition in youngsters with CF. Pediatr Pulmonol. 2013;49:960–70.eight.Sly PD, Brennan S, Gangell C, et al. Lung illness at analysis in infants with cystic fibrosis detected by new child screening. Am J Respir Crit Care Med. 2009;180:146–52.9.Meyerholz DK, Stoltz DA, Namati E, et al. Lack of cystic fibrosis transmembrane conductance regulator perform produces abnormalities in tracheal growth in neonatal pigs and younger youngsters. Am J Respir Crit Care Med. 2010;182:1251–61.10.Sturgess J, Imrie J. Quantitative analysis of the event of tracheal submucosal glands in infants with cystic fibrosis and management infants. Am J Pathol. 1982;106:303–11.11.Rogers CS, Stoltz DA, Meyerholz DK, et al. Disruption of the CFTR gene produces a mannequin of cystic fibrosis in new child pigs. Science. 2008;321:1837–41.12.Stoltz DA, Rokhlina T, Ernst SE, et al. Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs. J Clin Make investments. 2013;123:2685–93.13.Ostedgaard LS, Meyerholz DK, Chen JH, et al. The DeltaF508 mutation causes CFTR misprocessing and cystic fibrosis-like illness in pigs. Sci Transl Med. 2011;three:74ra24.14.Adam RJ, Michalski AS, Bauer C, et al. Air trapping and airflow obstruction in new child cystic fibrosis piglets. Am J Respir Crit Care Med. 2013;188:1434–41.15.Gibson-Corley KN, Olivier AK, Meyerholz DK. Ideas for legitimate histopathologic scoring in analysis. Vet Pathol. 2013;50:1007–15.16.Meyerholz DK, Lambertz AM, Reznikov LR, et al. Immunohistochemical detection of markers for translational research of lung illness in pigs and people. Toxicol Pathol. 2015;44:434–41.17.Burri PH. Fetal and postnatal growth of the lung. Annu Rev Physiol. 1984;46:617–28.18.Michoud MC, Robert R, Hassan M, et al. Function of the cystic fibrosis transmembrane conductance channel in human airway clean muscle. Am J Respir Cell Mol Biol. 2009;40:217–22.19.Li X, Tang XX, Vargas Buonfiglio LG, et al. Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host protection. Am J Physiol Lung Cell Mol Physiol. 2016;310:L670–9.20.Chang EH, Pezzulo AA, Meyerholz DK, et al. Sinus hypoplasia precedes sinus an infection in a porcine mannequin of cystic fibrosis. Laryngoscope. 2012;122:1898–905.21.Trapnell C, Williams BA, Pertea G, et al. Transcript meeting and quantification by RNA-Seq reveals unannotated transcripts and isoform switching throughout cell differentiation. Nat Biotechnol. 2010;28:511–5.22.RJ Adam MH Abou Alaiwa DC Bouzek et al. 2017 Postnatal airway development in cystic fibrosis piglets J Appl Physiol (1985) 123 526 33.23.Kiernan JA. Dyes and different colorants in microtechnique and biomedical analysis. Coloration Technol. 2006;122:1–21.24.Sanjai Ok, Kumarswamy J, Patil A, Papaiah L, Jayaram S, Krishnan L. Analysis and comparability of decalcification brokers on the human tooth. J Oral Maxillofac Pathol. 2012;16:222–7.25.Singer M, Morrison PR. The affect of pH, dye, and salt focus on the dye binding of modified and unmodified fibrin. J Biol Chem. 1948;175:133–45.26.Chen JH, Stoltz DA, Karp PH, et al. Lack of anion transport with out elevated sodium absorption characterizes new child porcine cystic fibrosis airway epithelia. Cell. 2010;143:911–23.27.McCray PB Jr., Reenstra WW, Louie E, Johnson J, Bettencourt JD, Bastacky J. Expression of CFTR and presence of cAMP-mediated fluid secretion in human fetal lung. Am J Physiol. 1992;262:L472–81.28.Pezzulo AA, Tang XX, Hoegger MJ, et al. Lowered airway floor pH impairs bacterial killing within the porcine cystic fibrosis lung. Nature. 2012;487:109–13.29.Shah VS, Meyerholz DK, Tang XX, et al. Airway acidification initiates host protection abnormalities in cystic fibrosis mice. Science. 2016;351:503–7.30.McCray PB Jr., Bettencourt JD, Bastacky J. Growing bronchopulmonary epithelium of the human fetus secretes fluid. Am J Physiol. 1992;262:L270–9.31.Graeff RW, Wang G, McCray PB Jr.. KGF and FGF-10 stimulate liquid secretion in human fetal lung. Pediatr Res. 1999;46:523–9.32.Bellusci S, Grindley J, Emoto H, Itoh N, Hogan BL. Fibroblast development issue 10 (FGF10) and branching morphogenesis within the embryonic mouse lung. Improvement. 1997;124:4867–78.33.Mailleux AA, Kelly R, Veltmaat JM, et al. Fgf10 expression identifies parabronchial clean muscle cell progenitors and is required for his or her entry into the sleek muscle cell lineage. Improvement. 2005;132:2157–66.34.Marcorelles P, Montier T, Gillet D, Lagarde N, Ferec C. Evolution of CFTR protein distribution in lung tissue from regular and CF human fetuses. Pediatr Pulmonol. 2007;42:1032–40.35.Tiddens HA, Donaldson SH, Rosenfeld M, Pare PD. Cystic fibrosis lung illness begins within the small airways: can we deal with it extra successfully? Pediatr Pulmonol. 2010;45:107–17.36.Trezise AE, Chambers JA, Wardle CJ, Gould S, Harris A. Expression of the cystic fibrosis gene in human foetal tissues. Hum Mol Genet. 1993;2:213–eight.37.Tizzano EF, Chitayat D, Buchwald M. Cell-specific localization of CFTR mRNA reveals developmentally regulated expression in human fetal tissues. Hum Mol Genet. 1993;2:219–24.38.Brennan SC, Wilkinson WJ, Tseng HE, et al. The extracellular calcium-sensing receptor regulates human fetal lung growth by way of CFTR. Sci Rep. 2016;6:21975.39.Clement R, Blanc P, Mauroy B, Sapin V, Douady S. Form self-regulation in early lung morphogenesis. PLoS ONE. 2012;7:e36925.40.Bonvin E, Le Rouzic P, Bernaudin JF, et al. Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice. J Physiol. 2008;586:3231–43.41.Stoltz DA, Meyerholz DK, Pezzulo AA, et al. Cystic fibrosis pigs develop lung illness and exhibit faulty bacterial eradication at start. Sci Transl Med. 2010;2:29ra31.42.Tuggle KL, Birket SE, Cui X, et al. Characterization of defects in ion transport and tissue growth in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats. PLoS ONE. 2014;9:e91253.43.Broackes-Carter FC, Mouchel N, Gill D, Hyde S, Bassett J, Harris A. Temporal regulation of CFTR expression throughout ovine lung growth: implications for CF gene remedy. Hum Mol Genet. 2002;11:125–31.44.Jesse NM, McCartney J, Feng X, Richards EM, Wooden CE, Keller-Wooden M. Expression of ENaC subunits, chloride channels, and aquaporins in ovine fetal lung: ontogeny of expression and results of altered fetal cortisol concentrations. Am J Physiol Regul Integr Comp Physiol. 2009;297:R453–61.45.McGrath SA, Basu A, Zeitlin PL. Cystic fibrosis gene and protein expression throughout fetal lung growth. Am J Respir Cell Mol Biol. 1993;eight:201–eight.46.Tebbutt SJ, Wardle CJ, Hill DF, Harris A. Molecular evaluation of the ovine cystic fibrosis transmembrane conductance regulator gene. Proc Natl Acad Sci USA. 1995;92:2293–7.47.Olver RE, Strang LB. Ion fluxes throughout the pulmonary epithelium and the secretion of lung liquid within the foetal lamb. J Physiol. 1974;241:327–57.48.Harding RHS. Lung development and maturation. In: Rodeck CHWM, editor. Fetal medication: fundamental science and scientific observe. 2nd ed. New York, NY: Elsevier. 2009. p. 133–46..49.Vilos GA, Liggins GC. Intrathoracic pressures in fetal sheep. J Dev Physiol. 1982;four:247–56.50.Liu M, Tanswell AK, Put up M. Mechanical force-induced sign transduction in lung cells. Am J Physiol. 1999;277:L667–83.51.Okiyoneda T, Veit G, Dekkers JF, et al. Mechanism-based corrector mixture restores DeltaF508-CFTR folding and performance. Nat Chem Biol. 2013;9:444–54.52.Dekkers JF, Wiegerinck CL, de Jonge HR, et al. A practical CFTR assay utilizing main cystic fibrosis intestinal organoids. Nat Med. 2013;19:939–45.53.Boj SF, Vonk AM, Statia M et al. Forskolin-induced swelling in intestinal organoids: an in vitro assay for assessing drug response in cystic fibrosis sufferers. J Vis Exp 2017;120:e55159.54.Zhang H, Sweezey NB, Kaplan F. LGL1 modulates proliferation, apoptosis, and migration of human fetal lung fibroblasts. Am J Physiol Lung Cell Mol Physiol. 2015;308:L391–402.55.Shannon JM, Hyatt BA. Epithelial−mesenchymal interactions within the growing lung. Annu Rev Physiol. 2004;66:625–45.56.Cook dinner DP, Rector MV, Bouzek DC, et al. CFTR in sarcoplasmic reticulum of airway clean muscle: implications for airway contractility. Am J Respir Crit Care Med. 2015;193:417–26.57.Colvin JS, White AC, Pratt SJ, Ornitz DM. Lung hypoplasia and neonatal demise in Fgf9-null mice determine this gene as a necessary regulator of lung mesenchyme. Improvement. 2001;128:2095–106.58.De Moerlooze L, Spencer-Dene B, Revest JM, Hajihosseini M, Rosewell I, Dickson C. An vital position for the IIIb isoform of fibroblast development issue receptor 2 (FGFR2) in mesenchymal-epithelial signalling throughout mouse organogenesis. Improvement. 2000;127:483–92.59.Abler LL, Mansour SL, Solar X. Conditional gene inactivation reveals roles for Fgf10 and Fgfr2 in establishing a traditional sample of epithelial branching within the mouse lung. Dev Dyn. 2009;238:1999–2013.60.Wong AP, Bear CE, Chin S, et al. Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing practical CFTR protein. Nat Biotechnol. 2012;30:876–82.61.Sekine Ok, Ohuchi H, Fujiwara M, et al. Fgf10 is crucial for limb and lung formation. Nat Genet. 1999;21:138–41.62.Ramasamy SK, Mailleux AA, Gupte VV, et al. Fgf10 dosage is essential for the amplification of epithelial cell progenitors and for the formation of a number of mesenchymal lineages throughout lung growth. Dev Biol. 2007;307:237–47.63.Sala FG, Del Ethical PM, Tiozzo C, et al. FGF10 controls the patterning of the tracheal cartilage rings by way of Shh. Improvement. 2011;138:273–82.64.Nedvetsky PI, Emmerson E, Finley JK, et al. Parasympathetic innervation regulates tubulogenesis within the growing salivary gland. Dev Cell. 2014;30:449–62.65.Rogers CS, Abraham WM, Brogden KA, et al. The porcine lung as a possible mannequin for cystic fibrosis. Am J Physiol Lung Cell Mol Physiol. 2008;295:L240–63.66.Cohen JC, Larson JE, Killeen E, Love D, Takemaru Ok. CFTR and Wnt/beta-catenin signaling in lung growth. BMC Dev Biol. 2008;eight:70.67.Gaillard D, Ruocco S, Lallemand A, Dalemans W, Hinnrasky J, Puchelle E. Immunohistochemical localization of cystic fibrosis transmembrane conductance regulator in human fetal airway and digestive mucosa. Pediatr Res. 1994;36:137–43.68.Crawford I, Maloney PC, Zeitlin PL, et al. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc Natl Acad Sci USA. 1991;88:9262–6.


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