IgA肾病异常糖基化IgA1分子的研究现状

IgA肾病是最常见的原发性肾小球疾病。研究表明沉积于肾小球系膜区的IgA主要为IgA1,IgA1分子的异常糖基化是导致IgA肾病发病的关键因素。糖基化酶缺乏、基因突变、免疫紊乱都可能导致IgA1异常糖基化的发生。异常糖基化IgA1分子可通过自我聚集或形成免疫复合物沉积于系膜区,进而刺激系膜细胞增殖、分泌系膜基质、细胞因子、趋化因子、生长因子等,导致肾小球炎性反应。对IgA1异常糖基化的深入研究有助于了解IgA肾病的发病机制并提供新的诊断与治疗方法。

Research Status of Aberrant Glycosylation of IgA1 in IgA Nephropathy

IgA nephropathy(IgAN),the most common form of primary glomerulonephritis worldwide,is dened by predominant IgA1 deposits in the glomerular 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.Aberrant glycosylation of IgA1 has a tendency to accumulate in glomerular mesangium by self-aggregation or immunecomplex formation.Glomerular mesangial cells exposed to immune complexes of these IgA1 can proliferate and secrete extracellular matrix com-ponents,cytokines,chemokines and growth factors promoting in?ammatory reactions in the glomeruli.The further studies on aberrant glycosylation of IgA1 would contribute to the understanding of IgA nephropathy and provide new diagnosis and therapy.