Epithelial Signaling and Transport Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD, USA.
EMBO J. 2019 Jan 15;38(2). doi: 10.15252/embj.2018101200. Epub 2018 Dec 20.
Coeliac disease (CD) is an inflammatory autoimmune disease caused by ingestion of gluten proteins, mainly gliadin. Undigested gliadin proline‐rich peptides trigger the innate and adaptive immune response, resulting in intestinal cell stress and damage. A new study by Villella (2019) addressing the unclear primary cause of intestinal cell stress reports that gliadin peptides inhibit the function of the chloride and bicarbonate channel CFTR, causing intestinal cell stress, which is sufficient to trigger CD symptoms. Notably, CFTR potentiators used to treat cystic fibrosis effectively rescue CFTR function and markedly ameliorate the pathology of coeliac disease.
乳糜泻(CD)是一种由摄入麸质蛋白(主要是麦胶蛋白)引起的炎症性自身免疫性疾病。未消化的麦胶蛋白脯氨酸丰富肽触发先天和适应性免疫反应,导致肠道细胞应激和损伤。Villella (2019 年)进行的一项新研究针对肠道细胞应激的不明确主要原因报告称,麦胶蛋白肽抑制氯离子和碳酸氢根通道 CFTR 的功能,导致肠道细胞应激,这足以引发 CD 症状。值得注意的是,用于治疗囊性纤维化的 CFTR 增强剂可有效恢复 CFTR 功能,并显著改善乳糜泻的病理。