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胶原蛋白在正常和纤维化的人类骨骼肌中的定位。

Collagen localization in normal and fibrotic human skeletal muscle.

作者信息

Foidart M, Foidart J M, Engel W K

出版信息

Arch Neurol. 1981 Mar;38(3):152-7. doi: 10.1001/archneur.1981.00510030046006.

Abstract

The distribution of types I to IV collagen, types I and III p-N collagen, and fibronectin in human skeletal muscle was studied by immunofluorescence using purified antibodies to those proteins. In normal muscle, types I and III collagen, types I and III p-N collagen, and fibronectin were localized in the endomysium and perimysium. Type IV collagen was restricted to basement membrane. Type II collagen was not present. In Duchenne's musclar dystrophy and dermatomyositis/polymyositis (DM/PM), the prominently increased endomysial and perimysial fibrosis consisted of types I and III collagen, types I and III p-N collagen, and fibronectin. In DM/PM, thickening of the walls of perimysial venular and arteriolar vessels was associated with accumulation of types I and III collagen, types I and III p-N collagen, and fibronectin, as well as type IV collagen. There was no disease-specific accumulation of collagen, p-N collagen, or fibronectin.

摘要

使用针对这些蛋白质的纯化抗体,通过免疫荧光法研究了I至IV型胶原蛋白、I型和III型对硝基胶原蛋白(p-N胶原蛋白)以及纤连蛋白在人体骨骼肌中的分布。在正常肌肉中,I型和III型胶原蛋白、I型和III型p-N胶原蛋白以及纤连蛋白定位于肌内膜和肌束膜。IV型胶原蛋白局限于基底膜。II型胶原蛋白不存在。在杜兴氏肌营养不良症和皮肌炎/多肌炎(DM/PM)中,肌内膜和肌束膜显著增加的纤维化由I型和III型胶原蛋白、I型和III型p-N胶原蛋白以及纤连蛋白组成。在DM/PM中,肌束膜小静脉和小动脉血管壁增厚与I型和III型胶原蛋白、I型和III型p-N胶原蛋白、纤连蛋白以及IV型胶原蛋白的积累有关。胶原蛋白、p-N胶原蛋白或纤连蛋白不存在疾病特异性积累。

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