Prevalence of HSP47 antigen and autoantibodies to HSP47 in the sera of patients with mixed connective tissue disease
Prevalence of HSP47 antigen and autoantibodies to HSP47 in the sera of patients with mixed connective tissue disease
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DOI：
10.1016/s0006-291x(03)00352-8
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发表时间：
2003-04-04
影响因子：
3.1
通讯作者：
Nagata, K
中科院分区：
文献类型：
--
作者：
Yokota, SI;Kubota, H;Nagata, K
47-kDa热休克蛋白（HSP 47）是一种内质网分子伴侣，有助于胶原分子的成熟，其表达已知在纤维化疾病的病变中上调。我们用酶联免疫吸附试验和免疫印迹分析检测了风湿性自身免疫性疾病患者血清中HSP 47蛋白和HSP 47自身抗体的水平，包括类风湿性关节炎、系统性红斑狼疮、干燥综合征和混合性结缔组织病（MCTD）。特发性肺纤维化（IPF）患者被评估为非自身免疫性纤维化疾病的一个例子。HSP 47抗原和自身抗体水平在风湿性自身免疫病患者的血清中显著升高，但在IPF患者的血清中不显著升高。MCTD患者的血清相对于健康对照显示出特别高的HSP 47抗原水平（1.99 +/-0.22vs 0. 41 +/-0.07ng/ml）。MCTD患者血清中HSP 47自身抗体水平也较高。这些结果表明，全身炎症的同时发生和HSP 47的上调导致HSP 47从纤维化病变泄漏到外周血中，并且泄漏的抗原诱导了高滴度的HSP 47自身抗体。高水平的HSP 47抗原和自身抗体可能是MCTD的有用血液标志物。(C)2003 Elsevier Science（美国）。All rights reserved.
The 47-kDa heat shock protein (HSP47) is an endoplasmic reticulum molecular chaperone that assists in the maturation of collagen molecules and whose expression is known to be upregulated in lesions of fibrotic diseases. We examined the levels of HSP47 protein and autoantibodies to HSP47 in the sera of patients with rheumatic autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus, Sjogren's syndrome, and mixed connective tissue disease (MCTD) by enzyme-linked immunosorbent assay and immunoblot analysis. Patients with idiopathic pulmonary fibrosis (IPF) were assessed as an example of non-autoimmune fibrotic disease. HSP47 antigen and autoantibody levels are significantly elevated in the sera of the rheumatic autoimmune disease patients, but not in the sera of the IPF patients. The sera of the MCTD patients showed particularly high levels of HSP47 antigen relative to healthy controls (1.99 +/- 0.22 vs 0.41 +/- 0.07 ng/ml). Autoantibodies to HSP47 were also in high levels in the sera of MCTD patients. These results suggest that simultaneous occurrence of systemic inflammation and upregulation of HSP47 caused leakage of HSP47 from fibrotic lesions into the peripheral blood, and the leaked antigen induced high titer of autoantibodies to HSP47. The high levels of HSP47 antigen and autoantibody may be useful blood markers of MCTD. (C) 2003 Elsevier Science (USA). All rights reserved.