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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: van der Werf, Christine S., Hsiao, Nai-Hua, Conroy, Siobhan, Paredes, Joana, Ribeiro, Ana S., Sribudiani, Yunia, Seruca, Raquel, Hofstra, Robert M. W., Westers, Helga, van IJzendoorn, Sven C. D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3584079/
https://www.ncbi.nlm.nih.gov/pubmed/23460781
http://dx.doi.org/10.1371/journal.pone.0054649
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author van der Werf, Christine S.
Hsiao, Nai-Hua
Conroy, Siobhan
Paredes, Joana
Ribeiro, Ana S.
Sribudiani, Yunia
Seruca, Raquel
Hofstra, Robert M. W.
Westers, Helga
van IJzendoorn, Sven C. D.
author_facet van der Werf, Christine S.
Hsiao, Nai-Hua
Conroy, Siobhan
Paredes, Joana
Ribeiro, Ana S.
Sribudiani, Yunia
Seruca, Raquel
Hofstra, Robert M. W.
Westers, Helga
van IJzendoorn, Sven C. D.
author_sort van der Werf, Christine S.
collection PubMed
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.
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spelling pubmed-35840792013-03-04 CLMP Is Essential for Intestinal Development, but Does Not Play a Key Role in Cellular Processes Involved in Intestinal Epithelial Development van der Werf, Christine S. Hsiao, Nai-Hua Conroy, Siobhan Paredes, Joana Ribeiro, Ana S. Sribudiani, Yunia Seruca, Raquel Hofstra, Robert M. W. Westers, Helga van IJzendoorn, Sven C. D. PLoS One Research Article 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. Public Library of Science 2013-02-27 /pmc/articles/PMC3584079/ /pubmed/23460781 http://dx.doi.org/10.1371/journal.pone.0054649 Text en © 2013 van der Werf et al http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Research Article
van der Werf, Christine S.
Hsiao, Nai-Hua
Conroy, Siobhan
Paredes, Joana
Ribeiro, Ana S.
Sribudiani, Yunia
Seruca, Raquel
Hofstra, Robert M. W.
Westers, Helga
van IJzendoorn, Sven C. D.
CLMP Is Essential for Intestinal Development, but Does Not Play a Key Role in Cellular Processes Involved in Intestinal Epithelial Development
title 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_short 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
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3584079/
https://www.ncbi.nlm.nih.gov/pubmed/23460781
http://dx.doi.org/10.1371/journal.pone.0054649
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