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Inositol 145-trisphosphate receptor type 1 autoantibodies in paraneoplastic and non-paraneoplastic peripheral neuropathy

机译:副肿瘤和非副肿瘤周围神经病中的145-三磷酸肌醇1型自身抗体

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摘要

BackgroundRecently, we described a novel autoantibody, anti-Sj/ITPR1-IgG, that targets the inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) in patients with cerebellar ataxia. However, ITPR1 is expressed not only by Purkinje cells but also in the anterior horn of the spinal cord, in the substantia gelatinosa and in the motor, sensory (including the dorsal root ganglia) and autonomic peripheral nervous system, suggesting that the clinical spectrum associated with autoimmunity to ITPR1 may be broader than initially thought. Here we report on serum autoantibodies to ITPR1 (up to 1:15,000) in three patients with (radiculo)polyneuropathy, which in two cases was associated with cancer (ITPR1-expressing adenocarcinoma of the lung, multiple myeloma), suggesting a paraneoplastic aetiology.
机译:背景技术最近,我们描述了一种新型的自身抗体,抗Sj / ITPR1-IgG,其针对小脑性共济失调患者的1、4、5-三磷酸三磷酸受体1(ITPR1)肌醇。但是,ITPR1不仅在浦肯野细胞中表达,而且在脊髓的前角,明胶性黑质以及运动,感觉(包括背根神经节)和自主神经末梢神经系统中表达,提示临床相关对ITPR1具有自身免疫力的患者可能比最初认为的要广泛。在这里,我们报告了3例(神经根)多发性神经病患者的ITPR1血清自身抗体(最高1:15,000),其中2例与癌症(ITPR1表达的肺腺癌,多发性骨髓瘤)有关,提示副肿瘤病因。

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