title: Defensins couple dysbiosis to primary immunodeficiency in Crohn's disease.
abstract: Antimicrobial peptides, including defensins, are essential effectors in host defence and in the maintenance of immune homeostasis. Clinical studies have linked the defective expression of both α- and β-defensin to the reduced killing of certain microorganisms by the intestinal mucosa of patients suffering from ileal and colonic Crohn's disease (CD), respectively. Only recently have the events leading to defective expression of defensins in CD been further elucidated, and are discussed herein. These events may account for CD-associated alterations in the microbiome and may subsequently precipitate the development of granulomatous inflammatory lesions in genetically-predisposed patients. We also address how these discoveries may pave the way for the development of a molecular medicine aimed at restoring gut barrier function in CD.
authors.affiliation: Institut Pasteur de Lille, Center for Infection and Immunity of Lille, F-59019 Lille, France. mathias.chamaillard@inserm.fr
authors.forename: Mathias
authors.initials: M
authors.lastname: Chamaillard
authors.forename: Rodrigue
authors.initials: R
authors.lastname: Dessein
mesh_terms: D000890:Anti-Infective Agents / Q000276:immunology*; D003424:Crohn Disease / Q000276:immunology* / Q000503:physiopathology; D023082:Defensins / Q000276:immunology*; D006801:Humans; D007107:Immune System / Q000276:immunology; D007153:Immunologic Deficiency Syndromes / Q000276:immunology* / Q000503:physiopathology; D007413:Intestinal Mucosa / Q000276:immunology / Q000382:microbiology / Q000502:physiology; D020033:Protein Isoforms / Q000276:immunology*
chemical_list: D000890:Anti-Infective Agents; D023082:Defensins; D020033:Protein Isoforms