Detection of anti-mitochondrial antibodies by immunoprecipitation in patients with systemic sclerosis

Detection of anti-mitochondrial antibodies by immunoprecipitation in patients with systemic sclerosis
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DOI:
10.1016/j.jim.2017.10.001
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发表时间:
2018-01-01
影响因子:
2.2
通讯作者:
Selmi, Carlo
Selmi, Carlo
中科院分区:
医学4区
文献类型:
--
作者:
Ceribelli, Angela;Isailovic, Natasa;Selmi, Carlo

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目的:描述在意大利系统性硬化症(SSC)患者中发现的一种新的免疫沉淀模式,该模式对应于原发性胆管炎(PBC)中抗线粒体抗体(AMA)识别的丙酮酸脱氢酶抗原复合体。结果:在85份SSC血清中,有一组独特的75-50-40-34kD的蛋白质被发现,这是以前没有报道过的。经免疫沉淀-Western印迹鉴定为丙酮酸脱氢酶复合体的X/E3BP、E1α、E1β和E2/E3蛋白。从临床资料看,9/13(69%)的SSC患者常规间接免疫荧光法AMA阳性,7/13(54%)的患者被诊断为PBC,4/13(31%)的患者没有胆汁淤积的生化征象。免疫沉淀法检测的13例SSc和AMA患者中,12例SSC和AND-着丝粒抗体的皮肤型有限。结论:我们描述了15%的SSC患者中有4种蛋白质的模式,这是首次通过蛋白质免疫沉淀鉴定的。这种模式对应于抗丙酮酸脱氢酶抗原复合体的血清AMA,在解释蛋白质免疫沉淀结果时必须考虑到这一点。
Objective: To describe a new immunoprecipitation pattern identified in Italian patients affected by systemic sclerosis (SSc), corresponding to the pyruvate dehydrogenase antigen complex recognized by anti-mitochondrial antibodies (AMA) in primary biliary cholangitis (PBC).Methods: Autoantibodies in sera from 85 patients with SSc were tested by protein- and RNA-immunoprecipitation. Inununoprecipitation-Western blot was used to determine the identified proteins, and medical records re-evaluated for liver function tests and PBC.Results: In 13/85 (15%) SSc sera, a unique set of 75-50-40-34 kD proteins that had not been previously reported, was noted. The four proteins were identified as the proteins X/E3BP, E1 alpha, E1 beta, and E2/E3 of the pyruvate dehydrogenase antigen complex by immunoprecipitation-Western blot. From clinical record evaluation, 9/13 (69%) SSc patients with this new pattern were positive for AMA by routine indirect immunofluorescence, and 7/13 (54%) had a diagnosis of PBC, while 4/13 (31%) manifested no biochemical signs of cholestasis. Twelve of 13 patients with SSc and AMA by immunoprecipitation have a limited cutaneous form of SSc and and-centromere antibodies.Conclusions: We describe a pattern of 4 proteins in 15% of SSc patients, identified for the first time by protein-immunoprecipitation. This pattern corresponds to serum AMA against the pyruvate dehydrogenase antigen complex and it must be considered in the interpretation of protein-immunoprecipitation results.