CHANGES IN SURFACE-MORPHOLOGY AND BASAL LAMINA OF CULTURED MUSCLE-CELLS FROM DUCHENNE MUSCULAR-DYSTROPHY PATIENTS
CHANGES IN SURFACE-MORPHOLOGY AND BASAL LAMINA OF CULTURED MUSCLE-CELLS FROM DUCHENNE MUSCULAR-DYSTROPHY PATIENTS
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DOI：
10.1016/0022-510x(90)90118-7
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发表时间：
1990-01-01
影响因子：
4.4
通讯作者：
FARDEAU, M
中科院分区：
文献类型：
--
作者：
DELAPORTE, C;DAUTREAUX, B;FARDEAU, M
杜氏肌营养不良症（DMD）患者培养的肌细胞从单核阶段显示出改变的生长：形态异常，细胞分裂减少，群体分裂数减少和融合延迟。在此基础上，应用扫描电镜观察细胞形态和表面形态，并对4种基底膜成分（IV型胶原、层粘连蛋白、纤维连接蛋白、硫酸乙酰肝素蛋白多糖（HSPG））进行免疫细胞化学定位。将融合指数高于65%的8个DMD肌肉培养物与来自10个年龄匹配的对照的肌肉培养物进行比较。营养不良的肌细胞观察到以下结果：（1）细胞表面光滑，有一些细长的细胞突起和锚定延伸;（2）IV型胶原和层粘连蛋白的分布不均匀，有大片（IV型胶原）或网状（层粘连蛋白）;（3）相反，纤连蛋白和HSPG水平明显降低。这些分子没有形成网络，而是排列在粗丝和补丁。细胞表面形态可能与纤维连接蛋白和HSPG的减少有关，这可能反映了基底层更普遍的减少。这些发现可以解释营养不良培养的细胞的低存活率和成肌细胞的延迟融合。尽管这些异常在成肌细胞融合后最大程度地表达，但它们已经存在于单核细胞中，并且它们与DMD中的主要缺陷（即，缺乏肌营养不良蛋白）的联系现在必须澄清。
Cultured muscle cells from Duchenne muscular dystrophy (DMD) patients showed altered growth from the mononucleated stage: abnormal morphology, decreased adhesiveness, reduced number of population doublings and delayed fusion. On the basis of these findings, a study was undertaken to observe cell shape and surface morphology by scanning electron microscopy and to the define the immunocytochemical localization of 4 basal lamina components (type IV collagen, laminin, fibronectin heparan sulfate proteoglycan (HSPG)). Eight DMD muscle cultures with fusion indices higher than 65% were compared to muscle cultures from 10 age-matched control. The following results were noted for the dystrophic muscle cells: (1) the cell surface was smooth with a few slender cell processes and anchorage extensions; (2) distribution of type IV collagen and laminin was heterogenous, with large patches (type IV collagen) or a reticulum (laminin); (3) in contrast, fibronectin and HSPG levels were clearly decreased. These molecules did not form network but rather were arranged in thick filaments and patches. Cell surface morphology may be related to the decreases in fibronectin and HSPG, which could reflect a more general decrease in basal lamina. Such findings could explain the low adhesiveness of the cells from dystrophic cultures and the delayed fusion of myoblasts. Although these abnormalities were maximally expressed after myoblast fusion, they were already present in mononucleated cells and their connection with the primary defect in DMD, i.e, lack of dystrophin, must now be clarified.