In:
PLOS ONE, Public Library of Science (PLoS), Vol. 15, No. 11 ( 2020-11-17), p. e0242167-
Abstract:
Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are relatively frequently occurring foregut malformations. EA/TEF is thought to have a strong genetic component. Not much is known regarding the biological processes disturbed or which cell type is affected in patients. This hampers the detection of the responsible culprits (genetic or environmental) for the origin of these congenital anatomical malformations. Therefore, we examined gene expression patterns in the TEF and compared them to the patterns in esophageal, tracheal and lung control samples. We studied tissue organization and key proteins using immunohistochemistry. There were clear differences between TEF and control samples. Based on the number of differentially expressed genes as well as histological characteristics, TEFs were most similar to normal esophagus. The BMP-signaling pathway, actin cytoskeleton and extracellular matrix pathways are downregulated in TEF. Genes involved in smooth muscle contraction are overexpressed in TEF compared to esophagus as well as trachea. These enriched pathways indicate myofibroblast activated fibrosis. TEF represents a specific tissue type with large contributions of intestinal smooth muscle cells and neurons. All major cell types present in esophagus are present—albeit often structurally disorganized—in TEF, indicating that its etiology should not be sought in cell fate specification.
Type of Medium:
Online Resource
ISSN:
1932-6203
DOI:
10.1371/journal.pone.0242167
DOI:
10.1371/journal.pone.0242167.g001
DOI:
10.1371/journal.pone.0242167.g002
DOI:
10.1371/journal.pone.0242167.g003
DOI:
10.1371/journal.pone.0242167.t001
DOI:
10.1371/journal.pone.0242167.t002
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10.1371/journal.pone.0242167.t003
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10.1371/journal.pone.0242167.t004
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10.1371/journal.pone.0242167.t005
DOI:
10.1371/journal.pone.0242167.s001
DOI:
10.1371/journal.pone.0242167.s002
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10.1371/journal.pone.0242167.s003
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10.1371/journal.pone.0242167.s004
DOI:
10.1371/journal.pone.0242167.s005
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10.1371/journal.pone.0242167.s006
DOI:
10.1371/journal.pone.0242167.s007
DOI:
10.1371/journal.pone.0242167.s008
DOI:
10.1371/journal.pone.0242167.s009
DOI:
10.1371/journal.pone.0242167.s010
DOI:
10.1371/journal.pone.0242167.s011
DOI:
10.1371/journal.pone.0242167.s012
DOI:
10.1371/journal.pone.0242167.s013
DOI:
10.1371/journal.pone.0242167.s014
DOI:
10.1371/journal.pone.0242167.s015
DOI:
10.1371/journal.pone.0242167.r001
DOI:
10.1371/journal.pone.0242167.r002
DOI:
10.1371/journal.pone.0242167.r003
DOI:
10.1371/journal.pone.0242167.r004
Language:
English
Publisher:
Public Library of Science (PLoS)
Publication Date:
2020
detail.hit.zdb_id:
2267670-3
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