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在杜氏肌营养不良症、炎性肌病及其他神经肌肉疾病中,肌纤维上HLA ABC I类抗原表达增加。

Increased expression of HLA ABC class I antigens by muscle fibres in Duchenne muscular dystrophy, inflammatory myopathy, and other neuromuscular disorders.

作者信息

Appleyard S T, Dunn M J, Dubowitz V, Rose M L

出版信息

Lancet. 1985 Feb 16;1(8425):361-3. doi: 10.1016/s0140-6736(85)91384-4.

Abstract

The distribution of HLA ABC class I antigens in human skeletal muscle obtained by needle biopsy was investigated by means of a monoclonal antibody (W6/32) and an immunoperoxidase technique. Five samples from normal individuals and twenty-nine from patients with various neuromuscular disorders were examined. Normal muscle fibres and those from patients with congenital muscular dystrophy expressed little or no class I antigens, whereas muscle fibres of patients with myositis and various X-linked muscular dystrophies showed consistently strong expression. In other neuromuscular diseases expression was more variable. The presence of class I antigens on diseased muscle fibres may render them susceptible to cytotoxic T cells; these antigens may thus have an important role in the destruction of muscle fibres.

摘要

采用单克隆抗体(W6/32)和免疫过氧化物酶技术,研究了通过针吸活检获得的人骨骼肌中HLA ABC I类抗原的分布。检查了5例正常个体的样本和29例患有各种神经肌肉疾病患者的样本。正常肌纤维以及先天性肌营养不良患者的肌纤维表达很少或不表达I类抗原,而肌炎和各种X连锁肌营养不良患者的肌纤维则始终显示出强烈的表达。在其他神经肌肉疾病中,表达变化更大。患病肌纤维上I类抗原的存在可能使它们易受细胞毒性T细胞的攻击;因此,这些抗原可能在肌纤维的破坏中起重要作用。

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