Clinical and serological features of patients with autoantibodies to GW/P bodies
Clinical and serological features of patients with autoantibodies to GW/P bodies
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DOI：
10.1016/j.clim.2007.07.016
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发表时间：
2007-12-01
影响因子：
8.6
通讯作者：
Fritzler, Marvin J.
中科院分区：
文献类型：
--
作者：
Bhanji, Rahima A.;Eystathioy, Theophany;Fritzler, Marvin J.
GW小体是一种独特的细胞质结构，参与信使RNA(MRNA)加工和RNA干扰(RNAi)。GWB包括信使核糖核酸、核糖核酸诱导沉默复合体的组成部分、微RNA、ArgAerte蛋白、GE-L/Hedls蛋白以及其他与信使核糖核酸降解有关的酶。本研究的目的是确定与55例抗GWB自身抗体患者的血清反应的靶GWB自身抗原，并确定与这些抗体相关的临床特征。可寻址激光珠免疫分析(ALBIA)和重组蛋白免疫沉淀分析表明，该队列抗GWB血清中的自身抗体针对GE-1/Hedls(58%)、GW182(40%)和Ago2(16%)。GWB自身抗体针对的表位包括Ago2的N端和包含GE-1/Hedls区域的核定位信号(NLS)。42例患者有临床资料，其中女性39例，平均年龄61岁。最常见的临床表现是神经系统症状(即共济失调、运动神经病和感觉神经病)(33%)，干燥综合征(SjS)(31%)，其余的有各种其他诊断，包括系统性红斑狼疮(SLE)、类风湿性关节炎(RA)和原发性胆汁性肝硬化(PBC)。此外，44%的抗GWB抗体患者对Ro52有反应性。这些研究表明，GE-1是抗GWB血清的常见靶点，GWB队列中的大多数患者都有干燥综合征和神经系统疾病。(C)2007 Elsevier Inc.保留所有权利。
GW bodies (GWBs) are unique cytoplasmic structures involved in messenger RNA (mRNA) processing and RNA interference (RNAi). GWBs contain mRNA, components of the RNA-induced silencing complex (RISC), microRNA (miRNA), Argonaute proteins, the Ge-l/Hedls protein and other enzymes involving mRNA degradation. The objective of this study was to identify the target GWB autoantigens reactive with 55 sera from patients with anti-GWB autoantibodies and to identify clinical features associated with these antibodies. Analysis by addressable laser bead immunoassay (ALBIA) and immunoprecipitation of recombinant proteins indicated that autoantibodies in this cohort of anti-GWB sera were directed against Ge-1 /Hedls (58%), GW182 (40%) and Ago2 (16%). GWB autoantibodies targeted epitopes that included the N-terminus of Ago2 and the nuclear localization signal (NLS) containing region of Ge-1/Hedls. Clinical data were available on 42 patients of which 39 were female and the mean age was 61 years. The most common clinical presentations were neurological symptoms (i.e. ataxia, motor and sensory neuropathy) (33%), Sjogren's syndrome (SjS) (31%) and the remainder had a variety of other diagnoses that included systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and primary biliary cirrhosis (PBC). Moreover, 44% of patients with anti-GWB antibodies had reactivity to Ro52. These studies indicate that Ge-1 is a common target of anti-GWB sera and the majority of patients in a GWB cohort had SjS and neurological disease. (c) 2007 Elsevier Inc. All rights reserved.