title: Cystic fibrosis: evidence for gut inflammation.
abstract: Cystic fibrosis (CF) gut manifestations are predominantly secondary to cystic fibrosis transmembrane regulator protein (CFTR) dysfunction. The CFTR gene is expressed throughout the intestinal tract. Because the intestine is difficult to assess in humans, there exists a lack of data on the underlying mechanisms of intestinal dysfunction. A more tractable approach involves the use of mouse models of CF, created by gene targeting techniques, to describe the consequences of CFTR dysfunction in the intestinal tissues, including mucus accumulation, disturbed motility, small bowel bacterial overgrowth and inflammation with altered innate immune responses, that are likely to be interrelated. We will focus on the latter. Recently, in people with CF, even in the absence of overt gastrointestinal symptoms, chronic intestinal inflammation and abnormal balance of the microbiota have been evidenced. Because chronic gut inflammation may be a driver for systemic inflammation, the prevention and control of intestinal inflammation represents a promising research strategy.
authors.affiliation: Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Center, Université Paris 7, 75019 Paris, France. Electronic address: anne.munck@rdb.aphp.fr.
authors.forename: Anne
authors.initials: A
authors.lastname: Munck
mesh_terms: D000818:Animals; D003550:Cystic Fibrosis / Q000378:metabolism / Q000473:pathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D004195:Disease Models, Animal; D005759:Gastroenteritis / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D051379:Mice
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Cystic fibrosis; Gut; Immune response; Inflammation