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

Hindawi Publishing Corporation Clinical and Developmental Immunology Volume 2011, Article ID 470803, 7 pages doi:10.1155/2011/470803 Review Article Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy Mototsugu Tanaka,1 George Seki,1 Tomonosuke Someya,2 Michio Nagata,3 and Toshiro Fujita1 1 Department of Internal Medicine, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, Japan 2 Department of Pediatrics, Juntendo University School of Medicine, Tokyo 113-8431, Japan 3 Molecular Pathology, Biomolecular and Integrated Medical Sciences, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Ibaraki 305-8575, Japan Correspondence should be addressed to George Seki, georgeseki-tky@umin.ac.jp Received 13 October 2010; Revised 3 December 2010; Accepted 6 January 2011 Academic Editor: Jiri Mestecky Copyright © 2011 Mototsugu Tanaka et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. 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 transplantatio