title: Aberrantly glycosylated IgA1 as a factor in the pathogenesis of IgA nephropathy.
abstract: Predominant or codominant immunoglobulin (Ig) A deposition in the glomerular mesangium characterizes IgA nephropathy (IgAN). Accumulated glomerular IgA is limited to the IgA1 subclass and usually galactose-deficient. This underglycosylated IgA may play an important role in the pathogenesis of IgAN. Recently, antibodies against galactose-deficient IgA1 were found to be well associated with the development of IgAN. Several therapeutic strategies based on corticosteroids or other immunosuppressive agents have been shown to at least partially suppress the progression of IgAN. On the other hand, several case reports of kidney transplantation or acquired IgA deficiency uncovered a remarkable ability of human kidney to remove mesangial IgA deposition, resulting in the long-term stabilization of kidney function. Continuous exposure to circulating immune complexes containing aberrantly glycosylated IgA1 and sequential immune response seems to be essential in the disease progression of IgAN. Removal of mesangial IgA deposition may be a challenging, but fundamental approach in the treatment of IgAN.
authors.affiliation: Department of Internal Medicine, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, Japan.
authors.forename: Mototsugu
authors.initials: M
authors.lastname: Tanaka
authors.forename: George
authors.initials: G
authors.lastname: Seki
authors.forename: Tomonosuke
authors.initials: T
authors.lastname: Someya
authors.forename: Michio
authors.initials: M
authors.lastname: Nagata
authors.forename: Toshiro
authors.initials: T
authors.lastname: Fujita
mesh_terms: D000818:Animals; D005922:Glomerulonephritis, IGA / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D006031:Glycosylation; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use
chemical_list: D007070:Immunoglobulin A; D007166:Immunosuppressive Agents