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β4-Galactosyltransferase Deficiency and IgA Nephropathy

  • Masahide Asano

Abstract

Immunoglobulin A nephropathy (IgAN) is the most common glomerulonephritis worldwide, and about 40% of the patients progress to renal failure 20 years after the onset of the disease in Japan. IgAN is characterized by glomerular mesangial expansion and IgA deposition. However, the pathological molecular mechanisms remain to be fully elucidated. Pathological roles for the abnormal serum IgA, especially abnormal galactosylation and sialylation of O-glycans of IgA1, have been proposed. We have reported that β1,4-galactosyltransferase-I-deficient (β4GalT-I-/-) mice spontaneously developed human IgAN-like disease with IgA deposition and expanded mesangial matrix (Nishie et al. 2007). This is the first report to demonstrate that genetic remodeling of protein glycosylation causes IgAN.

Keywords

Mesangial Matrix IgAN Patient Selectin Ligand Mesangial Matrix Expansion Human IgAN 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Reference

  1. Nishie T, Miyaishi O, Azuma H, Kameyama A, Naruse C, Hashimoto N, Yokoyama H, Narimatsu H, Wada T, Asano M (2007) Development of immunoglobulin A nephropathy-like disease in β-1,4-galactosyltransferase-I deficient mice. Am J Pathol 170:447–459PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2008

Authors and Affiliations

  • Masahide Asano
    • 1
  1. 1.Advanced Science Research CenterKanazawa UniversityKanazawaJapan

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