eng Science and Education Publishing International Journal of Celiac Disease 2328-3955 2014-09-17 2 3 89 92 10.12691/ijcd-2-3-5 IJCD2014235 article Erna Sziksz sziksz.erna@med.semmelweis-univ.hu 1 2 Apor Veres-Székely 2 2 Domonkos Pap 2 Andrea Fekete 2 3 Gábor Veres 3 Tivadar Tulassay 3 3 Attila Szabó 3 Ádám Vannay 3 3 MTA-SE, Pediatrics and Nephrology Research Group, H-1083, Budapest, Hungary Celiac disease (CD) is the most common autoimmune enteropathy in the western world caused by the intolerance to gluten in genetically predisposed individuals. CD is characterized by a remarkable rearrangement of the mucosal architecture, in which process myofibroblasts play a crucial role. Myofibroblasts (intestinal subepithelial myofibroblasts and interstitial cells of Cajal) are the most represented mesenchymal cell types in the gut mucosa and are involved in a broad range of biological processes including growth, mucosal protection, repair, inflammation and fibrosis. Myofibroblasts actively contribute to the mucosal changes in CD due to their ability to produce an excessive amount of extracellular matrix and basement membrane components (e.g. collagens, fibronectin, and specific enzymes including tissue transglutaminases) and through the expression of matrix metalloproteinases (MMPs) and tissue inhibitors of matrix metalloproteinases (TIMPs). The enhanced production of ECM components and MMPs and the altered shape and motility of myofibroblasts in the duodenal mucosa of patients with CD suggest that myofibroblasts may play an essential role in the pathogenesis of CD. http://pubs.sciepub.com/ijcd/2/3/5/ijcd-2-3-5.pdf celiac disease myofibroblast extracellular matrix tissue transglutaminase matrix metalloproteinase