Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children.

American Journal of Respiratory and Critical Care Medicine
David K MeyerholzM J Welsh

Abstract

Although airway abnormalities are common in patients with cystic fibrosis (CF), it is unknown whether they are all secondary to postnatal infection and inflammation, which characterize the disease. To learn whether loss of the cystic fibrosis transmembrane conductance regulator (CFTR) might affect major airways early in life, before the onset of inflammation and infection. We studied newborn CFTR⁻(/)⁻ pig trachea, using computed tomography (CT) scans, pathology, and morphometry. We retrospectively analyzed trachea CT scans in young children with CF and also previously published data of infants with CF. We discovered three abnormalities in the porcine CF trachea. First, the trachea and mainstem bronchi had a uniformly small caliber and cross-sections of trachea were less circular than in controls. Second, trachealis smooth muscle had an altered bundle orientation and increased transcripts in a smooth muscle gene set. Third, submucosal gland units occurred with similar frequency in the mucosa of CF and control airways, but CF submucosal glands were hypoplastic and had global reductions in tissue-specific transcripts. To learn whether any of these changes occurred in young patients with CF, we examined CT scans from children 2 yea...Continue Reading

References

Aug 1, 1991·The American Journal of Physiology·W T Gerthoffer
Dec 1, 1982·European Journal of Pediatrics·C W ChowL M Taussig
May 1, 1993·American Journal of Respiratory Cell and Molecular Biology·P B McCray
Mar 1, 1997·Experimental Lung Research·M D Infeld
Oct 12, 1999·The American Journal of Physiology·S T BallardA Crews
Dec 16, 2000·Journal of Anatomy·H K ChoiJ H Widdicombe
Feb 28, 2002·Respiratory Medicine·D A GronebergK F Chung
Mar 29, 2002·Pediatric Pulmonology·Michael Robinson, Peter T B Bye
Aug 7, 2002·Clinical Reviews in Allergy & Immunology·Miles Weinberger
Oct 9, 2002·The Journal of Biological Chemistry·Nam Soo JooJeffrey J Wine
Nov 8, 2002·American Journal of Respiratory and Critical Care Medicine·Sarath C RanganathanJanet Stocks
Feb 5, 2004·The Journal of Pediatrics·Frederick R LongRobert G Castile
Dec 15, 2004·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Danieli SalinasA S Verkman
Mar 3, 2005·Thorax·S R HaysP G Woodruff
May 13, 2005·The New England Journal of Medicine·Steven M RoweEric J Sorscher
Jul 30, 2005·American Journal of Respiratory and Critical Care Medicine·Tanya M MartínezRobert S Tepper
Aug 23, 2005·Proceedings of the American Thoracic Society·Jeffrey J Wine, Nam Soo Joo
Oct 4, 2005·Proceedings of the National Academy of Sciences of the United States of America·Aravind SubramanianJill P Mesirov
Jan 18, 2006·The Journal of Biological Chemistry·Nam Soo JooJeffrey J Wine
Apr 15, 2006·American Journal of Respiratory Cell and Molecular Biology·Jie PanHerman Yeger
Aug 19, 2006·American Journal of Respiratory and Critical Care Medicine·Steven R Hays, John V Fahy
Aug 25, 2006·Thorax·Pierre-Régis BurgelJay A Nadel
Aug 30, 2006·Respiratory Research·Clarisse VandebrouckFrédéric Becq
Sep 29, 2006·The European Respiratory Journal·N Sánchez-MoraE Alvarez-Fernández
Nov 23, 2006·Nature Medicine·Seok H YunBrett E Bouma
Jan 27, 2007·American Journal of Respiratory and Critical Care Medicine·Markus O HenkeBruce K Rubin
Aug 21, 2007·Respiratory Physiology & Neurobiology·Stephen T Ballard, Domenico Spadafora
Jan 26, 2008·American Journal of Respiratory and Critical Care Medicine·Nicolas RegameyJane C Davies
Jul 1, 2008·Developmental Biology·Jason R RockBrian D Harfe
Aug 16, 2008·American Journal of Respiratory Cell and Molecular Biology·Anthony J FischerPaul B McCray
Sep 2, 2008·American Journal of Respiratory Cell and Molecular Biology·Marie-Claire MichoudJames G Martin
Sep 23, 2008·Cell·Björn Christian SchroederLily Yeh Jan
Sep 27, 2008·Science·Christopher S RogersMichael J Welsh
Oct 15, 2008·Annual Review of Physiology·Omar Tliba, Reynold A Panettieri
Oct 18, 2008·Gastrointestinal Endoscopy·Melissa J SuterGuillermo J Tearney
Jan 28, 2009·PloS One·Robert C De LisleRacquel Sewell

❮ Previous
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Citations

Dec 16, 2011·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·N KlymiukE Wolf
May 25, 2011·Pflügers Archiv : European journal of physiology·Karl KunzelmannRainer Schreiber
Dec 7, 2013·Annals of Biomedical Engineering·Maged AwadallaDavid A Stoltz
Aug 4, 2012·Journal of Aerosol Medicine and Pulmonary Drug Delivery·Felix Ratjen
Mar 18, 2011·Science Translational Medicine·Lynda S OstedgaardDavid A Stoltz
Jul 17, 2012·American Journal of Physiology. Lung Cellular and Molecular Physiology·James F Collawn, Sadis Matalon
Jun 23, 2011·American Journal of Respiratory and Critical Care Medicine·Peter J Mogayzel, Patrick A Flume
Apr 26, 2013·American Journal of Respiratory Cell and Molecular Biology·Maria D I ManuntaStephen L Hart
Aug 27, 2010·The Journal of Clinical Investigation·Nam Soo JooJeffrey J Wine
May 17, 2013·The Journal of Clinical Investigation·David A StoltzMichael J Welsh
Dec 30, 2011·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Stephanie PlogLars Mundhenk
Oct 31, 2013·American Journal of Respiratory and Critical Care Medicine·Ryan J AdamDavid A Stoltz
Nov 13, 2013·Proceedings of the National Academy of Sciences of the United States of America·Elizabeth A HinesXin Sun
Jan 9, 2014·Physiological Reports·Helen L WallaceTheodor Burdyga
Nov 5, 2013·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Jinny J GuoRobert D Roghair
Jan 30, 2014·Proceedings of the National Academy of Sciences of the United States of America·Mark J HoeggerMichael J Welsh
Jan 14, 2011·Annual Review of Medicine·Melissa A Ashlock, Eric R Olson
Feb 22, 2012·Respiratory Care·S Rodrigo BurgueteJay I Peters
Feb 5, 2013·American Journal of Physiology. Lung Cellular and Molecular Physiology·Alessandra Livraghi-ButricoBarbara R Grubb
Nov 20, 2015·Expert Review of Respiratory Medicine·John J BrewingtonJohn P Clancy
Oct 22, 2015·American Journal of Respiratory and Critical Care Medicine·Daniel P CookDavid A Stoltz
Feb 10, 2016·Clinics in Chest Medicine·John Brewington, J P Clancy
Feb 2, 2016·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Hagit LevineHannah Blau
Oct 12, 2011·The Journal of Physiology·Peying Fong
Jan 26, 2016·The Journal of Clinical Investigation·Xiao Xiao TangMichael J Welsh
Jan 1, 2016·Medical & Biological Engineering & Computing·Stephen M HumphriesEmily M DeBoer
Sep 11, 2012·Computerized Medical Imaging and Graphics : the Official Journal of the Computerized Medical Imaging Society·Christian BauerReinhard R Beichel
May 30, 2012·Clinics in Chest Medicine·Jason LoboPeadar G Noone
Feb 14, 2012·Respiratory Physiology & Neurobiology·Nicolas LeboulangerBrigitte Fauroux
Nov 21, 2015·Asian Journal of Andrology·Sylvie BretonBongki Kim
Sep 12, 2015·Jornal de pediatria·Jose Dirceu Ribeiro, Gilberto Bueno Fischer
Aug 1, 2012·Reproduction in Domestic Animals = Zuchthygiene·A Bähr, E Wolf
Sep 14, 2012·Developmental Dynamics : an Official Publication of the American Association of Anatomists·Erin J PlosaLawrence S Prince
Jun 21, 2014·Respirology : Official Journal of the Asian Pacific Society of Respirology·Kathryn A Ramsey, Sarath Ranganathan
Apr 2, 2015·The Laryngoscope·Amit DiwakarDavid A Stoltz
Oct 30, 2013·Pediatric Pulmonology·Anthony J FischerTimothy D Starner
Aug 21, 2012·Journal of Histotechnology·Katherine N Gibson-CorleyDavid K Meyerholz
Oct 22, 2014·Journal of Histotechnology·Christine M HochstedlerDavid K Meyerholz
Sep 4, 2015·American Journal of Respiratory Cell and Molecular Biology·T Idil Apak EvansJohn F Engelhardt

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