{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T17:50:50Z","timestamp":1786729850218,"version":"build-2736575974"},"reference-count":63,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2023,3,16]],"date-time":"2023-03-16T00:00:00Z","timestamp":1678924800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Max-Delbr\u00fcck-Center (Berlin, Germany)","award":["GO865\/13-1"],"award-info":[{"award-number":["GO865\/13-1"]}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["GO865\/13-1"],"award-info":[{"award-number":["GO865\/13-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The immunoglobulin-like cell adhesion molecule CLMP is a member of the CAR family of cell adhesion proteins and is implicated in human congenital short-bowel syndrome (CSBS). CSBS is a rare but very severe disease for which no cure is currently available. In this review, we compare data from human CSBS patients and a mouse knockout model. These data indicate that CSBS is characterized by a defect in intestinal elongation during embryonic development and impaired peristalsis. The latter is driven by uncoordinated calcium signaling via gap junctions, which is linked to a reduction in connexin43 and 45 levels in the circumferential smooth muscle layer of the intestine. Furthermore, we discuss how mutations in the CLMP gene affect other organs and tissues, including the ureter. Here, the absence of CLMP produces a severe bilateral hydronephrosis\u2014also caused by a reduced level of connexin43 and associated uncoordinated calcium signaling via gap junctions.<\/jats:p>","DOI":"10.3390\/ijms24065719","type":"journal-article","created":{"date-parts":[[2023,3,17]],"date-time":"2023-03-17T03:33:57Z","timestamp":1679024037000},"page":"5719","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["The IgSF Cell Adhesion Protein CLMP and Congenital Short Bowel Syndrome (CSBS)"],"prefix":"10.3390","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5351-613X","authenticated-orcid":false,"given":"Fritz G.","family":"Rathjen","sequence":"first","affiliation":[{"name":"Max-Delbr\u00fcck-Center for Molecular Medicine, Robert-R\u00f6ssle-Str. 10, 13092 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ren\u00e9","family":"J\u00fcttner","sequence":"additional","affiliation":[{"name":"Max-Delbr\u00fcck-Center for Molecular Medicine, Robert-R\u00f6ssle-Str. 10, 13092 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/S0016-5085(69)80074-0","article-title":"Short small intestine associated with malrotation: A newly described congenital cause of intestinal malabsorption","volume":"56","author":"Hamilton","year":"1969","journal-title":"Gastroenterology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2352","DOI":"10.1016\/j.bbadis.2015.08.007","article-title":"Congenital Short Bowel Syndrome: From clinical and genetic diagnosis to the molecular mechanisms involved in intestinal elongation","volume":"1852","author":"Halim","year":"2015","journal-title":"Biochim. 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