IgA1 aberrant glycosylation in the pathogenesis of IgA nephropathy: an overivew / 生物医学工程学杂志

ABSTRACT

IgA nephropathy is the most common form of primary glomerulonephritis which mainly accounts for the development of end-stage renal diseases. It is characterized by deposits of IgA1 in mesangium. The pathogenesis of IgA nephropathy is complicated. Moreover, there is a wide range of clinical features and variable histomorphologies in the diagnosed cases of IgA nephropathy. It was demonstrated that the galactose-deficient of IgA1 O-glycan chains led IgA1 to self-aggregation and eventual deposition in mesangium. Abnormality of glycosyltransferases, genetic mutation and immunologic disorder were involved in the aberrant glycosylation of IgA1 which was recognized as the key etiopathogenisis of IgA nephropathy. However, the exact source and the pathogenic mechanism of aberrantly glycosylated IgA1 remain obscure. The further studies on aberrant O-glycosylation of IgA1 would contribute to the understanding of IgA nephropathy and provide new therapeutical strategy.