A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease

ABSTRACT: Intestinal handling of dietary proteins usually prevents local inflammatory and immune responses and promotes oral tolerance. However, in ∼1% of the world population, gluten proteins from wheat and related cereals trigger an HLA DQ2/8-restricted TH1 immune and antibody response leading to celiac disease. Prior epithelial stress and innate immune activation are essential for breaking oral tolerance to the gluten component gliadin. How gliadin subverts host intestinal mucosal defenses remains elusive. Here we show that the -gliadin-derived LGQQQPFPPQQPY peptide (P31-43) inhibits the function of cystic fibrosis transmembrane conductance regulator (CFTR), an anion channel pivotal for epithelial adaptation to cell-autonomous or environmental stress. P31-43 binds to, and reduces ATPase activity

SUBMITTER: Prof. Luigi Maiuri 

PROVIDER: S-SCDT-EMBOJ-2018-100101 | biostudies-other |

REPOSITORIES: biostudies-other