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Although neuromuscular\u2010glial\u2010ICC abnormalities represent the main pathogenetic mechanism, the pathophysiology of CIPO remains poorly understood. Intestinal epithelial and vascular endothelial barrier (IEVB) abnormalities can contribute to neuroepithelial changes by allowing passage of harmful substances.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>To test retrospectively whether IEVB defects occur in patients with CIPO, we measured the jejunal protein expression of the major tight junction (TJ) components. CIPO patients were subdivided according to gut neuromuscular histopathology: apparently normal (AN); with inflammation (INF); or with degenerative alterations (DEG). The presence of occludin\/claudin oligomers (index of TJ assembly), the amount of occludin, claudin\u20104, and <jats:italic>zonula occludens<\/jats:italic>\u20101 (ZO\u20101), and the expression of vasoactive intestinal polypeptide (VIP) and glial fibrillary acidic protein (GFAP) immunoreactivities were evaluated on jejunal full\u2010thickness biopsies using Western blot.<\/jats:p><\/jats:sec><jats:sec><jats:title>Key Results<\/jats:title><jats:p>Oligomers were absent in the 73% of CIPO. Total occludin decreased in CIPO with AN and INF changes. Claudin\u20104 was upregulated in CIPO with INF and DEG features. ZO\u20101 and VIP expression decreased selectively in DEG group. GFAP increased in CIPO regardless the histopathological phenotype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions &amp; Inferences<\/jats:title><jats:p>The absence of oligomers demonstrated in our study suggests that IEBV is altered in CIPO. The mechanism leading to oligomerization is occludin\u2010dependent in AN and INF, whereas is ZO\u20101\u2010dependent in DEG. Our study provides support to IEVB abnormalities contributing to CIPO clinical and histopathological features.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nmo.13652","type":"journal-article","created":{"date-parts":[[2019,5,30]],"date-time":"2019-05-30T00:39:28Z","timestamp":1559176768000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Gut epithelial and vascular barrier abnormalities in patients with chronic intestinal pseudo\u2010obstruction"],"prefix":"10.1111","volume":"31","author":[{"given":"Elisa","family":"Boschetti","sequence":"first","affiliation":[{"name":"Department of Medical and Surgical Sciences University of Bologna Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Accarino","sequence":"additional","affiliation":[{"name":"Digestive System Research Unit, University Hospital Vall d'Hebron Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades 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