PML Nuclear Body Component Sp140 Is a Novel Autoantigen in Primary Biliary Cirrhosis
PML Nuclear Body Component Sp140 Is a Novel Autoantigen in Primary Biliary Cirrhosis
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DOI：
10.1038/ajg.2009.596
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发表时间：
2010-01-01
影响因子：
9.8
通讯作者：
Muratori, Paolo
中科院分区：
文献类型：
--
作者：
Granito, Alessandro;Yang, Wei-Hong;Muratori, Paolo
提示：一些原发性胆汁性肝硬化（PBC）患者有抗核抗体（ANA）。这些ANA包括“多核点”（MND）染色模式，靶向早幼粒细胞白血病蛋白（PML）核体（NB）组分，如“斑点100-kD”蛋白（Sp100）和PML。一种新的PML NB蛋白，命名为Sp140，使用PBC患者的血清进行鉴定。本研究的目的是分析PBC patients.METHODS对Sp140蛋白的免疫应答：我们研究了135例PBC患者和157例病理对照1型自身免疫性肝炎，原发性硬化性胆管炎，系统性红斑狼疮。我们采用间接免疫荧光法和表达Sp140的神经母细胞瘤细胞系检测抗Sp140抗体，并采用市售的免疫印迹法检测抗Sp100和抗PML抗体。结果：20例PBC患者（15%）存在抗Sp140抗体，而对照组无此抗体，抗线粒体抗体（AMA）阴性病例中的频率更高（53 vs. 9%，P < 0.05）。
OBJECTIVES: Some patients with primary biliary cirrhosis (PBC) have antinuclear antibodies (ANAs). These ANAs include the "multiple nuclear dots" (MND) staining pattern, targeting promyelocytic leukemia protein (PML) nuclear body (NB) components, such as "speckled 100-kD" protein (Sp100) and PML. A new PML NB protein, designated as Sp140, was identified using serum from a PBC patient. The aim of this study was to analyze the immune response against Sp140 protein in PBC patients.METHODS: We studied 135 PBC patients and 157 pathological controls with type 1 autoimmune hepatitis, primary sclerosing cholangitis, and systemic lupus erythematosus. We used indirect immunofluorescence and a neuroblastoma cell line expressing Sp140 for detecting anti-Sp140 antibodies, and a commercially available immunoblot for detecting anti-Sp100 and anti-PML antibodies.RESULTS: Anti-Sp140 antibodies were present in 20 (15%) PBC patients but not in control samples, with a higher frequency in antimitochondrial antibody (AMA)-negative cases (53 vs. 9%, P