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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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) |
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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 |
work_keys_str_mv |
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