Mucociliary Transport in Healthy and Cystic Fibrosis Pig Airways

Yuliang Xie; Lynda Ostedgaard; Mahmoud H Abou Alaiwa; Lin Lu; Anthony J Fischer; David A Stoltz

doi:10.1513/AnnalsATS.201805-308AW

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Mucociliary Transport in Healthy and Cystic Fibrosis Pig Airways

Journal article

Open access

Peer reviewed

Mucociliary Transport in Healthy and Cystic Fibrosis Pig Airways

Yuliang Xie, Lynda Ostedgaard, Mahmoud H Abou Alaiwa, Lin Lu, Anthony J Fischer and David A Stoltz

Annals of the American Thoracic Society, Vol.15(Suppl 3), pp.S171-S176

11/2018

DOI: 10.1513/AnnalsATS.201805-308AW

PMCID: PMC6322029

PMID: 30431346

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Abstract

Cystic fibrosis (CF) lung disease is the major cause of morbidity and mortality in people with CF. Abnormal mucociliary transport has been the leading hypothesis for the underlying pathogenesis of CF airway disease. However, this has been difficult to investigate at very early time points. A porcine CF model, which recapitulates many features of CF disease in humans, enables studies to be performed in non-CF and CF pigs on the day that they are born. In newborn CF pigs, we found that under basal conditions, mucociliary transport rates in non-CF and CF pigs are similar. However, after cholinergic stimulation, which stimulates submucosal gland secretion, particles become stuck in the CF airways owing to a failure of mucus strands to release from submucosal glands. In this review, we summarize these recent discoveries and also discuss the morphology, composition, and function of mucins in the porcine lung.

Animals, Newborn

Animals

Cystic Fibrosis - metabolism

Swine

Cystic Fibrosis - physiopathology

Mucociliary Clearance - physiology

Mucus - metabolism

Respiratory Mucosa - physiology

Cystic Fibrosis - etiology

Disease Models, Animal

Details

Title: Subtitle: Mucociliary Transport in Healthy and Cystic Fibrosis Pig Airways
Creators: Yuliang Xie - 1 Department of Internal Medicine
Lynda Ostedgaard - 1 Department of Internal Medicine
Mahmoud H Abou Alaiwa - 1 Department of Internal Medicine
Lin Lu - 1 Department of Internal Medicine
Anthony J Fischer - 2 Department of Pediatrics
David A Stoltz - 5 Pappajohn Biomedical Institute, University of Iowa, Iowa City, Iowa
Resource Type: Journal article
Publication Details: Annals of the American Thoracic Society, Vol.15(Suppl 3), pp.S171-S176
DOI: 10.1513/AnnalsATS.201805-308AW
PMID: 30431346
PMCID: PMC6322029
NLM abbreviation: Ann Am Thorac Soc
ISSN: 2325-6621
eISSN: 2325-6621
Publisher: American Thoracic Society
Grant note: P30 ES005605 / NIEHS NIH HHS P01 HL091842 / NHLBI NIH HHS K08 HL136927 / NHLBI NIH HHS K08 HL135433 / NHLBI NIH HHS T32 HL007638 / NHLBI NIH HHS R01 HL136813 / NHLBI NIH HHS P01 HL051670 / NHLBI NIH HHS L40 HL134155 / NHLBI NIH HHS P30 DK054759 / NIDDK NIH HHS DP2 HL117744 / NHLBI NIH HHS
Language: English
Date published: 11/2018
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Pulmonary Medicine; Stead Family Department of Pediatrics; Internal Medicine
Record Identifier: 9984093597102771

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