[Pathogenesis of Berger's disease: recent advances on the involvement of immunoglobulin A and their receptors].
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.
Monteiro
Renato C
RC
Inserm EMI-U 0225, UFR de Médecine Xavier Bichat, 16, rue Henri-Huchard, 75018 Paris, France. monteiro@bichat.inserm.fr
Leroy
Valérie
V
Launay
Pierre
P
Moura
Ivan Cruz
IC
Arcos-Fajardo
Michelle
M
Benhamou
Marc
M
Haddad
Elie
E
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
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