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

Laboratory Investigation; a Journal of Technical Methods and Pathology
David K MeyerholzPaul B McCray

Abstract

Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function causes cystic fibrosis (CF), predisposing the lungs to chronic infection and inflammation. In young infants with CF, structural airway defects are increasingly recognized before the onset of significant lung disease, which suggests a developmental origin and a possible role in lung disease pathogenesis. The role(s) of CFTR in lung development is unclear and developmental studies in humans with CF are not feasible. Young CF pigs have structural airway changes and develop spontaneous postnatal lung disease similar to humans; therefore, we studied lung development in the pig model (non-CF and CF). CF trachea and proximal airways had structural lesions detectable as early as pseudoglandular development. At this early developmental stage, budding CF airways had smaller, hypo-distended lumens compared to non-CF airways. Non-CF lung explants exhibited airway lumen distension in response to forskolin/IBMX as well as to fibroblast growth factor (FGF)-10, consistent with CFTR-dependent anion transport/secretion, but this was lacking in CF airways. We studied primary pig airway epithelial cell cultures and found that FGF10 increased cellular proliferation (non-CF ...Continue Reading

References

Oct 15, 1991·Proceedings of the National Academy of Sciences of the United States of America·I CrawfordC F Higgins
Jan 1, 1984·Annual Review of Physiology·P H Burri
Mar 14, 1995·Proceedings of the National Academy of Sciences of the United States of America·S J TebbuttA Harris
Feb 1, 1993·American Journal of Respiratory Cell and Molecular Biology·S A McGrathP L Zeitlin
Mar 1, 1993·Human Molecular Genetics·A E TreziseA Harris
Jan 23, 1999·Nature Genetics·K SekineS Kato
Dec 14, 1999·Pediatric Research·R W GraeffP B McCray
Jan 26, 2002·Human Molecular Genetics·Fiona C Broackes-CarterAnn Harris
Feb 24, 2004·Annual Review of Physiology·John M Shannon, Brian A Hyatt
May 13, 2005·The New England Journal of Medicine·Steven M RoweEric J Sorscher
Sep 29, 2007·Pediatric Pulmonology·Pascale MarcorellesClaude Ferec
May 20, 2008·American Journal of Physiology. Lung Cellular and Molecular Physiology·Christopher S RogersMichael J Welsh
Jul 8, 2008·BMC Developmental Biology·J Craig CohenKen-Ichi Takemaru
Sep 2, 2008·American Journal of Respiratory Cell and Molecular Biology·Marie-Claire MichoudJames G Martin
Sep 27, 2008·Science·Christopher S RogersMichael J Welsh
Apr 18, 2009·American Journal of Respiratory and Critical Care Medicine·Peter D SlyUNKNOWN Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST-CF)
Jun 12, 2009·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·Nathan M JesseMaureen Keller-Wood
Jul 21, 2009·Developmental Dynamics : an Official Publication of the American Association of Anatomists·Lisa L AblerXin Sun
Jan 19, 2010·Pediatric Pulmonology·Harm A W M TiddensPeter D Paré
Apr 30, 2010·Science Translational Medicine·David A StoltzMichael J Welsh
Jul 14, 2010·American Journal of Respiratory and Critical Care Medicine·David K MeyerholzMichael J Welsh
Dec 15, 2010·Development·Frédéric G SalaSaverio Bellusci
Mar 18, 2011·Science Translational Medicine·Lynda S OstedgaardDavid A Stoltz
May 23, 2012·PloS One·Raphaël ClémentStéphane Douady
Jun 20, 2012·The Laryngoscope·Eugene H ChangJoseph Zabner
Aug 28, 2012·Journal of Oral and Maxillofacial Pathology : JOMFP·Karpagaselvi SanjaiLakshmi Krishnan
Apr 6, 2013·Veterinary Pathology·K N Gibson-CorleyD K Meyerholz
May 15, 2013·Nature Chemical Biology·Tsukasa OkiyonedaGergely L Lukacs
May 17, 2013·The Journal of Clinical Investigation·David A StoltzMichael J Welsh
Jun 4, 2013·Nature Medicine·Johanna F DekkersJeffrey M Beekman
Oct 30, 2013·Pediatric Pulmonology·Anthony J FischerTimothy D Starner
Oct 31, 2013·American Journal of Respiratory and Critical Care Medicine·Ryan J AdamDavid A Stoltz
Aug 28, 2014·Developmental Cell·Pavel I NedvetskySarah M Knox
Dec 7, 2014·American Journal of Physiology. Lung Cellular and Molecular Physiology·Hui ZhangFeige Kaplan
Jan 22, 2015·The New England Journal of Medicine·David A StoltzMichael J Welsh
Oct 12, 2015·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Donald R VanDevanterPaul S Hodgkins
Oct 22, 2015·American Journal of Respiratory and Critical Care Medicine·Daniel P CookDavid A Stoltz
Oct 30, 2015·Toxicologic Pathology·David K MeyerholzDavid A Stoltz
Jan 24, 2016·American Journal of Physiology. Lung Cellular and Molecular Physiology·Xiaopeng LiJoseph Zabner

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Citations

Apr 2, 2019·Frontiers in Genetics·Soula DanopoulosDenise Al Alam
Jun 1, 2018·Laboratory Investigation; a Journal of Technical Methods and Pathology·David K Meyerholz, Amanda P Beck
Oct 26, 2018·BMC Research Notes·David K MeyerholzHeather A Flaherty
Nov 16, 2018·Frontiers in Genetics·Lawrence S Prince
Feb 5, 2019·ILAR Journal·David K Meyerholz, Amanda P Beck
Mar 5, 2020·Cell and Tissue Research·Amanda P Beck, David K Meyerholz
May 22, 2020·American Journal of Respiratory Cell and Molecular Biology·Rhianna E LeeScott H Randell
May 6, 2020·International Journal of Molecular Sciences·Margarida D AmaralInes Pankonien
Jul 29, 2020·Laboratory Investigation; a Journal of Technical Methods and Pathology·Keyan ZareiDavid A Stoltz
Oct 28, 2020·Cell Death & Disease·Margarida C QuaresmaMargarida D Amaral
Dec 12, 2020·The European Respiratory Journal·Jonathan L GillanRobert D Gray
Jun 23, 2021·Respiratory Physiology & Neurobiology·Emily M DeBoerUNKNOWN AREST CF
Jul 22, 2021·Physiological Reports·Keyan ZareiDavid A Stoltz
Nov 25, 2021·Science Translational Medicine·Joan K LunneyChaohui Dai
Dec 21, 2021·Frontiers in Cell and Developmental Biology·Alice E StantonCeleste M Nelson

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Methods Mentioned

BETA
PCR
biopsy

Software Mentioned

Ensembl
TopHat
Cuffdiff
CellSens
ImageJ

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