Defective galactosylation and clearance of IgA1 molecules as a possible etiopathogenic factor in IgA nephropathy.
Defective galactosylation and clearance of IgA1 molecules as a possible etiopathogenic factor in IgA nephropathy.
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IgA1 分子的半乳糖基化和清除缺陷可能是 IgA 肾病的致病因素。
DOI：
10.1159/000422410
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发表时间：
1993
影响因子：
--
通讯作者：
Jackson,S
中科院分区：
文献类型：
--
作者：
Mestecky,J;Tomana,M;Crowley-Nowick,PA;Moldoveanu,Z;Julian,BA;Jackson,S
已经在患有广泛疾病的患者中检测到升高的IgA 1、IgA 1-类风湿因子（RF）和含IgA 1的免疫复合物（IgA 1-IC）的血浆水平，所述疾病包括伊加肾病（IgAN）、获得性免疫缺陷综合征（AIDS）、疱疹样皮炎（DH）、过敏性紫癜（HSP）、系统性红斑狼疮（SLE）、酒精性肝病、类风湿性关节炎（RA）、其他疾病[1-10]尽管在IgAN的肾小球系膜和/或HSP和DH患者的皮肤中特征性地发现了组织沉积物，但它们并不总是在AIDS患者的组织中得到证实（4，11）。已经提出，多克隆或抗原特异性IgAl的过度产生和/或其清除缺陷可能参与IgAN的发病机制[1，2，12-14]。然而，由于过度刺激而产生的IgA 1过量不太可能是IgA 1-IC和组织中此类复合物沉积的原因：多发性骨髓瘤中极高的伊加血浆水平（高达50 g伊加/l）不会导致组织沉积，并且IgA-IC很少见到。特别引人注目的是IgAN和AIDS的差异：虽然明显不同的病因，但它们具有共同的特征，如血清IgA 1水平升高，IgA 1-RF和IgA 1-IC的存在，它们显然由相同的组分组成（IgA 1-IgG-C3）;
Elevated plasma levels of IgA1, IgA1-rheumatoid factor (RF), and IgA1-containing immune complexes (IgA1-IC) have been detected in patients with a broad spectrum of diseases including IgA nephropathy (IgAN), acquired immune deficiency syndrome (AIDS), dermatitis herpetiformis (DH), Henoch-Schönlein purpura (HSP), systemic lupus erythe-matosus (SLE), alcoholic liver disease, rheumatoid arthritis (RA), and other diseases [1-10]. Although, tissue deposits are characteristically found in the glomerular mesangium in IgAN and/or in the skin of HSP and DH patients, they cannot always be demonstrated in tissues of patients with AIDS (4, 11).It has been proposed that overproduction of polyclonal or antigen-specific IgAl and/or its defective clearance may be involved in the pathogenesis of IgAN [1, 2, 12-14]. However, mere overproduction of IgA1 due to excessive stimulation is an unlikely cause of IgA1-IC and deposition of such complexes in tissues: extremely high plasma levels of IgA (up to 50 g IgA/l) in multiple myeloma does not result in tissue deposition and IgA-IC are seen only rarely. Particularly striking are differences in IgAN and AIDS: although obviously of different etiology, they share common features such as increased serum levels of IgA1, the presence of IgA1-RF and of IgA1-IC that apparently are composed of identical components (IgA1-IgG-C3); how-