title: CLMP is essential for intestinal development, but does not play a key role in cellular processes involved in intestinal epithelial development.
abstract: 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.
authors: Department of Genetics, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands.
authors: Christine S
authors: CS
authors: van der Werf
authors: Nai-Hua
authors: NH
authors: Hsiao
authors: Siobhan
authors: S
authors: Conroy
authors: Joana
authors: J
authors: Paredes
authors: Ana S
authors: AS
authors: Ribeiro
authors: Yunia
authors: Y
authors: Sribudiani
authors: Raquel
authors: R
authors: Seruca
authors: Robert M W
authors: RM
authors: Hofstra
authors: Helga
authors: H
authors: Westers
authors: Sven C D
authors: SC
authors: van Ijzendoorn
mesh_terms: D000818:Animals; D016466:CHO Cells; D015815:Cell Adhesion Molecules / Q000378:metabolism; D002449:Cell Aggregation; D002465:Cell Movement; D049109:Cell Proliferation; D002470:Cell Survival; D062727:Coxsackie and Adenovirus Receptor-Like Membrane Protein / Q000378:metabolism*; D006224:Cricetinae; D003412:Cricetulus; D017097:Electric Impedance; D004847:Epithelial Cells / Q000166:cytology* / Q000378:metabolism*; D004848:Epithelium / Q000254:growth & development* / Q000378:metabolism; D006801:Humans; D007422:Intestines / Q000254:growth & development*; D050505:Mutant Proteins / Q000378:metabolism; D014161:Transduction, Genetic
chemical_list: C568771:CLMP protein, human; D015815:Cell Adhesion Molecules; D062727:Coxsackie and Adenovirus Receptor-Like Membrane Protein; D050505:Mutant Proteins