Inherited forms of IgA nephropathy

J Nephrol. Mar-Apr 2003;16(2):317-20.


Simplex and multiplex families with IgA nephropathy (IgAN) have been reported from several ethnic backgrounds, providing the strongest evidence of a role for genetic factors in pathogenesis of IgAN. From a phenotypic point of view, familial and sporadic IgAN cannot be differentiated, and the main clinical and histological features are similar. Traditionally, the case-control study design was employed to identify associations between particular candidate genes, for example, HLA antigens the uteroglobin gene and IgAN, giving conflicting results. Recently, a different approach, using linkage analysis, was undertaken by geneticists at Yale University. A 10-cM genome-wide screen was performed in 30 multiplex IgAN pedigrees, and one locus was mapped (IGAN-1) on chromosome 6q22-23. Future study will be focused on the identification of the gene underlying IGAN-1. This will enable us to understand the molecular pathogenetic basis of IgAN.

Publication types

  • Review

MeSH terms

  • Female
  • Genes, MHC Class II / genetics
  • Genetic Predisposition to Disease*
  • Glomerulonephritis, IGA / epidemiology
  • Glomerulonephritis, IGA / genetics*
  • HLA Antigens / genetics*
  • Humans
  • Incidence
  • Male
  • Molecular Biology
  • Pedigree
  • Phenotype
  • Polymorphism, Genetic*
  • Prognosis
  • Risk Factors


  • HLA Antigens