Muscle inflammation and MHC class I up-regulation in muscular dystrophy with lack of dysferlin: an immunopathological study
Muscle inflammation and MHC class I up-regulation in muscular dystrophy with lack of dysferlin: an immunopathological study
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DOI：
10.1016/s0165-5728(03)00255-8
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发表时间：
2003-09-01
影响因子：
3.3
通讯作者：
Mantegazza, R
中科院分区：
文献类型：
--
作者：
Confalonieri, P;Oliva, L;Mantegazza, R
肌肉炎症是炎性肌病的特征，但也发生在缺乏肌膜蛋白dysferlin的肌营养不良症中。我们定量了10名铁蛋白病患者肌肉中的炎症细胞和主要组织相容性复合体（MHC）表达。浸润细胞始终存在，但数量变化很大;巨噬细胞比T细胞更常见，T细胞毒性是不存在的，和MHC I类过表达的肌纤维。这些发现不同于多发性肌炎（PM），但与SJL/J小鼠（缺乏dysferlin）的发现非常相似，并强调了dysferlin缺乏与肌肉免疫系统异常之间的关系。(C)2003 Elsevier B. V.保留所有权利。
Muscle inflammation is characteristic of inflammatory myopathies but also occurs in muscular dystrophy with lack of the sarcolemmal protein dysferlin. We quantified inflammatory cells and major histocompatibility complex (MHC) expression in muscle from 10 patients with dysferlinopathy. Infiltrating cells were always present although numbers varied considerably; macrophages were more common than T cells, T cytotoxicity was absent, and MHC class I was overexpressed on muscle fibers. These findings differ from polymyositis (PM) but are closely similar to those in SJL/J mice (which lack dysferlin) and emphasize the relationship between absence of dysferlin and immune system abnormalities in muscle. (C) 2003 Elsevier B.V. All rights reserved.