The genetics and immunobiology of IgA nephropathy

J Clin Invest. 2014 Jun;124(6):2325-32. doi: 10.1172/JCI74475. Epub 2014 Jun 2.


IgA nephropathy (IgAN) represents the leading cause of kidney failure among East Asian populations and the most frequent form of primary glomerulonephritis among Europeans. Patients with IgAN develop characteristic IgA1-containing immune complexes that deposit in the glomerular mesangium, producing progressive kidney injury. Recent studies define IgAN as an autoimmune trait of complex architecture with a strong genetic determination. This Review summarizes new insights into the role of the O-glycosylation pathway, anti-glycan immune response, mucosal immunity, antigen processing and presentation, and the alternative complement pathway in the pathogenesis of IgAN.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Amino Acid Sequence
  • Antigen Presentation
  • Antigen-Antibody Complex / blood
  • Antigen-Antibody Complex / metabolism
  • Complement Pathway, Alternative
  • Genome-Wide Association Study
  • Glomerulonephritis, IGA / genetics*
  • Glomerulonephritis, IGA / immunology*
  • Glomerulonephritis, IGA / metabolism
  • Glycosylation
  • Humans
  • Immunity, Mucosal
  • Immunoglobulin A / blood
  • Immunoglobulin A / chemistry
  • Immunoglobulin A / genetics
  • Models, Genetic
  • Models, Immunological
  • Molecular Sequence Data
  • Molecular Structure


  • Antigen-Antibody Complex
  • Immunoglobulin A