Einerhand Alexandra W C, Renes Ingrid B, Makkink Mireille K, van der Sluis Maria, Büller Hans A, Dekker Jan
Laboratory of Paediatrics, Section Paediatric Gastroenterology and Nutrition, Room Ee 1571a, Erasmus Medical Centre and Sophia Children's Hospital, Dr Molewaterplein 50, 3015 GE Rotterdam, The Netherlands.
Eur J Gastroenterol Hepatol. 2002 Jul;14(7):757-65. doi: 10.1097/00042737-200207000-00008.
Inflammatory bowel disease (IBD) is characterized by a chronically inflamed mucosa of the gastrointestinal tract, caused by an underlying immune imbalance and triggered by luminal substances, including bacteria. Mucus forms a gel layer covering the gastrointestinal tract, acting as a semi-permeable barrier between the lumen and the epithelium. Mucins, the building blocks of the mucus gel, determine the thickness and properties of mucus. In IBD in humans, alterations in both membrane-bound and secretory mucins have been described involving genetic mutations in mucin genes, changes in mucin mRNA and protein levels, degree of glycosylation, sulphation, and degradation of mucins. As mucins are strategically positioned between the vulnerable mucosa and the bacterial contents of the bowel, changes in mucin structure and/or quantity probably influence their protective functions and therefore constitute possible aetiological factors in the pathogenesis of IBD. This hypothesis, however, is difficult to prove in humans. Animal models for IBD permit detailed analysis of those aspects of mucins necessary for protection against disease. These models revealed pertinent data as for how changes in mucins, in particular in MUC2, imposed by immunological or microbial factors, may contribute to the development and/or perpetuation of chronic IBD, and shed some light on possible strategies to counteract disease.
炎症性肠病（IBD）的特征是胃肠道黏膜长期发炎，由潜在的免疫失衡引起，并由包括细菌在内的管腔物质触发。黏液形成覆盖胃肠道的凝胶层，作为管腔与上皮之间的半透性屏障。黏蛋白是黏液凝胶的组成成分，决定了黏液的厚度和特性。在人类IBD中，已描述了膜结合黏蛋白和分泌型黏蛋白的改变，包括黏蛋白基因突变、黏蛋白mRNA和蛋白质水平的变化、糖基化程度、硫酸化以及黏蛋白的降解。由于黏蛋白处于脆弱的黏膜和肠道细菌内容物之间的关键位置，黏蛋白结构和/或数量的变化可能会影响其保护功能，因此可能是IBD发病机制中的病因因素。然而，这一假设在人类中很难得到证实。IBD动物模型允许对黏蛋白在预防疾病方面的必要方面进行详细分析。这些模型揭示了相关数据，即免疫或微生物因素引起的黏蛋白变化，特别是MUC2的变化，如何可能导致慢性IBD的发展和/或持续存在，并为对抗疾病的可能策略提供了一些线索。
