Progress in molecular and genetic studies of IgA nephropathy

J Clin Immunol. 2001 Sep;21(5):310-27. doi: 10.1023/a:1012284402054.


Several new findings emerged recently from biochemical, genetic, and molecular studies of patients with IgA nephropathy. It appears that immunoglobulin A1-secreting cells of IgA nephropathy patients produce increased amounts of aberrantly glycosylated IgA1 in which the O-linked glycans in the hinge region are deficient in the content of galactose. The galactose-deficient IgA1 in the circulation is recognized by naturally occurring antibodies with anti-glycan specificity, and immune complexes are formed. These circulating immune complexes escape hepatic degradation and eventually are deposited in the kidney mesangium. Resident mesangial cells bind the IgA-containing immune complexes with the involvement of a novel IgA receptor and become activated. A familial form of IgA nephropathy has been linked to chromosome 6q22-23. Recent progress in molecular analyses of IgA nephropathy thus defines this disease as an autoimmune process with a novel IgA mesangial receptor and certain genetically determined traits.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Chloride Channels / immunology
  • Galactose
  • Glomerular Mesangium / cytology
  • Glomerulonephritis, IGA / genetics
  • Glomerulonephritis, IGA / immunology*
  • Humans
  • Immunoglobulin A / biosynthesis
  • Immunoglobulin A / immunology*
  • Polysaccharides / biosynthesis


  • Chloride Channels
  • Immunoglobulin A
  • Polysaccharides
  • Galactose