Novak J, Julian B A, Tomana M, Mesteck J
Department of Microbiology, University of Alabama at Birmingham, 35294, USA.
J Clin Immunol. 2001 Sep;21(5):310-27. doi: 10.1023/a:1012284402054.
Several new findings emerged recently from biochemical, genetic, and molecular studies of patients with IgA nephropathy. It appears that immunoglobulin A1-secreting cells of IgA nephropathy patients produce increased amounts of aberrantly glycosylated IgA1 in which the O-linked glycans in the hinge region are deficient in the content of galactose. The galactose-deficient IgA1 in the circulation is recognized by naturally occurring antibodies with anti-glycan specificity, and immune complexes are formed. These circulating immune complexes escape hepatic degradation and eventually are deposited in the kidney mesangium. Resident mesangial cells bind the IgA-containing immune complexes with the involvement of a novel IgA receptor and become activated. A familial form of IgA nephropathy has been linked to chromosome 6q22-23. Recent progress in molecular analyses of IgA nephropathy thus defines this disease as an autoimmune process with a novel IgA mesangial receptor and certain genetically determined traits.
最近，对IgA肾病患者进行的生化、遗传和分子研究有了一些新发现。IgA肾病患者分泌免疫球蛋白A1的细胞似乎会产生数量增加的异常糖基化IgA1，其中铰链区的O-连接聚糖半乳糖含量不足。循环中的半乳糖缺陷型IgA1被具有抗聚糖特异性的天然抗体识别，并形成免疫复合物。这些循环免疫复合物逃避肝脏降解，最终沉积在肾小球系膜中。驻留的系膜细胞在一种新型IgA受体的参与下结合含IgA的免疫复合物并被激活。IgA肾病的家族形式与6号染色体q22-23相关。IgA肾病分子分析的最新进展因此将这种疾病定义为一种具有新型IgA系膜受体和某些遗传决定特征的自身免疫过程。
