Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy

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....

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Main Authors: Mototsugu Tanaka, George Seki, Tomonosuke Someya, Michio Nagata, Toshiro Fujita
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Clinical and Developmental Immunology
Online Access:http://dx.doi.org/10.1155/2011/470803
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author Mototsugu Tanaka
George Seki
Tomonosuke Someya
Michio Nagata
Toshiro Fujita
author_facet Mototsugu Tanaka
George Seki
Tomonosuke Someya
Michio Nagata
Toshiro Fujita
author_sort Mototsugu Tanaka
collection DOAJ
description 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.
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institution Kabale University
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spelling doaj-art-03bebccfb6b44244a656303b19080f972025-08-20T03:54:14ZengWileyClinical and Developmental Immunology1740-25221740-25302011-01-01201110.1155/2011/470803470803Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA NephropathyMototsugu Tanaka0George Seki1Tomonosuke Someya2Michio Nagata3Toshiro Fujita4Department of Internal Medicine, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, JapanDepartment of Internal Medicine, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, JapanDepartment of Pediatrics, Juntendo University School of Medicine, Tokyo 113-8431, JapanMolecular Pathology, Biomolecular and Integrated Medical Sciences, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Ibaraki 305-8575, JapanDepartment of Internal Medicine, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, JapanPredominant 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.http://dx.doi.org/10.1155/2011/470803
spellingShingle Mototsugu Tanaka
George Seki
Tomonosuke Someya
Michio Nagata
Toshiro Fujita
Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy
Clinical and Developmental Immunology
title Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy
title_full Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy
title_fullStr Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy
title_full_unstemmed Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy
title_short Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy
title_sort aberrantly glycosylated iga1 as a factor in the pathogenesis of iga nephropathy
url http://dx.doi.org/10.1155/2011/470803
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AT michionagata aberrantlyglycosylatediga1asafactorinthepathogenesisofiganephropathy
AT toshirofujita aberrantlyglycosylatediga1asafactorinthepathogenesisofiganephropathy