肌营养不良蛋白的同源物在杜氏肌营养不良症（DMD）和贝克型肌营养不良症（BMD）患者的血管膜上表达：免疫学证据。
A homologue of dystrophin is expressed at the blood vessel membrane of DMD and BMD patients: immunological evidence.
作者信息
Augier N, Boucraut J, Léger J, Anoal M, Nicholson L V, Voelkel M A, Léger J J, Pellissier J F
机构信息
INSERM U 300, Faculté de Pharmacie, Montpellier, France.
出版信息
J Neurol Sci. 1992 Feb;107(2):233-8. doi: 10.1016/0022-510x(92)90294-u.
Muscles from Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD) patients were analysed using monoclonal and polyclonal antibodies raised against different regions of the dystrophin molecule. On blot, two of the antibodies detected a protein of Mr 400K in muscle extracts from all patients, including a BMD patient with a deletion which spanned more than 40% of the central rod domain of the Xp21 encoded dystrophin. Immunocytochemical labelling of tissue sections from the same patients showed that the same two antibodies labelled a protein at the surface membrane of smooth muscle fibers in blood vessels of both BMD and DMD muscles. Thus we have demonstrated a 400K blood vessel-associated protein, which is immunologically homologous with dystrophin, for at least two epitopes from the carboxy terminal and the central rod domains must be encoded by another gene than the dystrophin gene.
使用针对肌营养不良蛋白分子不同区域产生的单克隆抗体和多克隆抗体，对来自贝克尔肌营养不良症（BMD）和杜兴肌营养不良症（DMD）患者的肌肉进行了分析。在印迹分析中，其中两种抗体在所有患者的肌肉提取物中检测到一种分子量为400K的蛋白质，包括一名BMD患者，其缺失跨越了Xp21编码的肌营养不良蛋白中央杆状结构域的40%以上。对同一患者组织切片的免疫细胞化学标记显示，相同的两种抗体在BMD和DMD肌肉血管中平滑肌纤维的表面膜上标记了一种蛋白质。因此，我们已经证明了一种与肌营养不良蛋白具有免疫同源性的400K血管相关蛋白，因为来自羧基末端和中央杆状结构域的至少两个表位必定由不同于肌营养不良蛋白基因的另一个基因编码。
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