CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.

Loss-of-function mutations in CLMP have been found in patients with Congenital Short Bowel Syndrome (CSBS), suggesting that its encoded protein plays a major role in intestinal development. CLMP is a membrane protein that co-localizes with tight junction proteins, but its function is largely unknown...

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Autores principales: Christine S van der Werf, Nai-Hua Hsiao, Siobhan Conroy, Joana Paredes, Ana S Ribeiro, Yunia Sribudiani, Raquel Seruca, Robert M W Hofstra, Helga Westers, Sven C D van Ijzendoorn
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Publicado: Public Library of Science (PLoS) 2013
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Acceso en línea:https://doaj.org/article/1f77282ca5de420986a7046b9d7ee00a
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spelling oai:doaj.org-article:1f77282ca5de420986a7046b9d7ee00a2021-11-18T07:55:55ZCLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.1932-620310.1371/journal.pone.0054649https://doaj.org/article/1f77282ca5de420986a7046b9d7ee00a2013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/23460781/?tool=EBIhttps://doaj.org/toc/1932-6203Loss-of-function mutations in CLMP have been found in patients with Congenital Short Bowel Syndrome (CSBS), suggesting that its encoded protein plays a major role in intestinal development. CLMP is a membrane protein that co-localizes with tight junction proteins, but its function is largely unknown. We expressed wild-type (WT)-CLMP and a mutant-CLMP (associated with CSBS) in human intestinal epithelial T84 cells that, as we show here, do not produce endogenous CLMP. We investigated the effects of WT-CLMP and mutant-CLMP proteins on key cellular processes that are important for intestinal epithelial development, including migration, proliferation, viability and transepithelial resistance. Our data showed that expression of WT-CLMP or mutant-CLMP does not affect any of these processes. Moreover, our aggregation assays in CHO cells show that CLMP does not act as a strong adhesion molecule. Thus, our data suggest that, in the in vitro model systems we used, the key processes involved in intestinal epithelial development appear to be unaffected by WT-CLMP or mutant-CLMP. Further research is needed to determine the role of CLMP in the development of the intestine.Christine S van der WerfNai-Hua HsiaoSiobhan ConroyJoana ParedesAna S RibeiroYunia SribudianiRaquel SerucaRobert M W HofstraHelga WestersSven C D van IjzendoornPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 2, p e54649 (2013)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Christine S van der Werf
Nai-Hua Hsiao
Siobhan Conroy
Joana Paredes
Ana S Ribeiro
Yunia Sribudiani
Raquel Seruca
Robert M W Hofstra
Helga Westers
Sven C D van Ijzendoorn
CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
description Loss-of-function mutations in CLMP have been found in patients with Congenital Short Bowel Syndrome (CSBS), suggesting that its encoded protein plays a major role in intestinal development. CLMP is a membrane protein that co-localizes with tight junction proteins, but its function is largely unknown. We expressed wild-type (WT)-CLMP and a mutant-CLMP (associated with CSBS) in human intestinal epithelial T84 cells that, as we show here, do not produce endogenous CLMP. We investigated the effects of WT-CLMP and mutant-CLMP proteins on key cellular processes that are important for intestinal epithelial development, including migration, proliferation, viability and transepithelial resistance. Our data showed that expression of WT-CLMP or mutant-CLMP does not affect any of these processes. Moreover, our aggregation assays in CHO cells show that CLMP does not act as a strong adhesion molecule. Thus, our data suggest that, in the in vitro model systems we used, the key processes involved in intestinal epithelial development appear to be unaffected by WT-CLMP or mutant-CLMP. Further research is needed to determine the role of CLMP in the development of the intestine.
format article
author Christine S van der Werf
Nai-Hua Hsiao
Siobhan Conroy
Joana Paredes
Ana S Ribeiro
Yunia Sribudiani
Raquel Seruca
Robert M W Hofstra
Helga Westers
Sven C D van Ijzendoorn
author_facet Christine S van der Werf
Nai-Hua Hsiao
Siobhan Conroy
Joana Paredes
Ana S Ribeiro
Yunia Sribudiani
Raquel Seruca
Robert M W Hofstra
Helga Westers
Sven C D van Ijzendoorn
author_sort Christine S van der Werf
title CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
title_short CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
title_full CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
title_fullStr CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
title_full_unstemmed CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
title_sort clmp is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
publisher Public Library of Science (PLoS)
publishDate 2013
url https://doaj.org/article/1f77282ca5de420986a7046b9d7ee00a
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