title: [Pathogenesis of Berger's disease: recent advances on the involvement of immunoglobulin A and their receptors].
abstract: Immunoglobulin A (IgA) nephropathy or Berger's disease is the most common form of primary glomerulonephritis in the world and one of the first cause of end-stage renal failure. IgA nephropathy is characterized by the accumulation in mesangial areas of immune complexes containing polymeric IgA1. While epidemiology and clinical studies of IgA nephropathy are well established, the mechanism(s) underlying disease development is poorly understood. The pathogenesis of this disease involves the deposition of polymeric and undergalactosylated IgA1 in the mesangium. Quantitative and structural changes of IgA1 play a key role in the development of the disease due to functional abnormalities of two IgA receptors: The FcalphaR (CD89) expressed by blood myeloid cells and the transferrin receptor (CD71) on mesangial cells. Abnormal IgA induce the release of soluble CD89 which is responsible for the formation of circulating IgA complexes. These complexes may be trapped by CD71 that is overexpressed on mesangial cells in IgA nephropathy patients allowing pathogenic IgA complex formation.
authors.affiliation: Inserm EMI-U 0225, UFR de Médecine Xavier Bichat, 16, rue Henri-Huchard, 75018 Paris, France. monteiro@bichat.inserm.fr
authors.forename: Renato C
authors.initials: RC
authors.lastname: Monteiro
authors.forename: Valérie
authors.initials: V
authors.lastname: Leroy
authors.forename: Pierre
authors.initials: P
authors.lastname: Launay
authors.forename: Ivan Cruz
authors.initials: IC
authors.lastname: Moura
authors.forename: Michelle
authors.initials: M
authors.lastname: Arcos-Fajardo
authors.forename: Marc
authors.initials: M
authors.lastname: Benhamou
authors.forename: Elie
authors.initials: E
authors.lastname: Haddad
mesh_terms: D015703:Antigens, CD / Q000276:immunology; D000944:Antigens, Differentiation, B-Lymphocyte / Q000276:immunology; D005922:Glomerulonephritis, IGA / Q000276:immunology* / Q000503:physiopathology*; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology* / Q000378:metabolism*; D011108:Polymers; D011961:Receptors, Fc / Q000276:immunology / Q000502:physiology*; D011990:Receptors, Transferrin
chemical_list: D015703:Antigens, CD; D000944:Antigens, Differentiation, B-Lymphocyte; C068126:CD71 antigen; C036832:Fc(alpha) receptor; C045281:IgA receptor; D007070:Immunoglobulin A; D011108:Polymers; D011961:Receptors, Fc; D011990:Receptors, Transferrin