Abstract
In 1992, the characteristic structure of O-linked oligosaccharides (O-glycans) in the IgA1 hinge and its possible aberrancy were simultaneously and independently proposed by Mesteckey et al., Allen et al., and our group at the International Congress of Nephrology (IgA Nephropathy 25th Year) held in Nancy, France. Since then, the aberrancy has been confirmed by several research groups and is suspected to play a role in the occurrence and/or the progression of IgAN.
In the serial investigations, it has been shown that the aberrant O-glycan structure in IgA1 may cause mesangial deposition of IgA in IgAN patients, but could not be proved that the aberrant IgA provokes tissue injury directly. Recently, the presence of antibody against aberrantly glycosylated IgA1 was elucidated.
This review illustrates the difficulty in elucidating the mechanisms of the development and aggravation of this disease.
