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Series GSE21071 Query DataSets for GSE21071
Status Public on Sep 17, 2010
Title Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth
Organism Sus scrofa
Experiment type Expression profiling by array
Summary Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF). However, understanding its pathogenesis has been hindered by lack of an animal model with characteristic features of CF. To overcome this problem, we recently generated pigs with targeted CFTR genes. We now report that within months of birth, CF pigs spontaneously develop hallmark features of CF lung disease including airway inflammation, remodeling, mucus accumulation, and infection. Their lungs contained multiple bacterial species, suggesting an equal opportunity host defense defect. In humans, the temporal and/or causal relationships between inflammation and infection have remained uncertain. To investigate these processes, we studied newborn pigs. Their lungs showed no inflammation, but were less often sterile than controls. Moreover, after intrapulmonary bacterial challenge, CF pigs failed to eradicate bacteria as effectively as wild- type pigs. These results suggest that impaired bacterial elimination is the pathogenic event that initiates a cascade of inflammation and pathology in CF lungs. Finding that CF pigs have a bacterial host defense defect within hours of birth provides an exciting opportunity to further investigate pathogenesis and to test therapeutic and preventive strategies before secondary consequences develop.
 
Overall design Pig model of human CF lung disease
 
Contributor(s) Stoltz DA, Meyerholz DK, Pezzulo AA, Ramachandran S, Rogan MP, Davis GJ, Hanfland RA, Wohlford-Lenane C, Dohrn CL, Bartlett JA, Nelson GA, Chang EH, Taft PJ, Ludwig PS, Estin M, Hornick EE, Launspach JL, Samuel M, Rokhlina T, Karp PH, Ostedgaard LS, Uc A, Starne TD, Horswill AR, Brogde KA, Prather RS, Richter SS, Shilyansky J, McCray PB, Zabner J, Welsh MJ
Citation(s) 20427821, 22763554
Submission date Mar 25, 2010
Last update date May 03, 2013
Contact name Shyam Ramachandran
E-mail(s) shyam-ramachandran@uiowa.edu
Phone 3193356933
Organization name Univ of Iowa
Department Pediatrics
Lab McCray Lab
Street address 240G EMRB
City Iowa City
ZIP/Postal code 52242
Country USA
 
Platforms (1)
GPL3533 [Porcine] Affymetrix Porcine Genome Array
Samples (40)
GSM526817 Trachea Newborn biological rep 1
GSM526818 Trachea Newborn biological rep 2
GSM526819 Trachea Newborn biological rep 3
Relations
BioProject PRJNA126737

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Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE21071_RAW.tar 90.9 Mb (http)(custom) TAR (of CEL)
Processed data included within Sample table

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