IgA nephropathy is the most common primary glomerulonephritis. It is caused by mesangial deposition of circulating immune complexes (IC) containing aberrantly glycosylated polymeric IgA1 (pIgA1).
Consequent mesangial proliferation and extracellular matrix expansion leads to a decrease or eventually loss of kidney function. This work summarizes our knowledge about mechanisms involved in pIgA1 synthesis, formation and properties of IC and perspectives of future disease-specific therapy.