Figarella-Branger D, Nedelec J, Pellissier J F, Boucraut J, Bianco N, Rougon G
Laboratoire d'Anatomie Pathologique et de Neuropathologie, Faculté de Médecine, Marseille, France.
J Neurol Sci. 1990 Aug;98(1):21-36. doi: 10.1016/0022-510x(90)90179-q.
Antibodies directed against neural cell adhesive molecules (NCAM) and in particular a monoclonal antibody recognizing polysialylated isoforms, were used to characterize the expression of these molecules in normal and diseased human muscles. Normal subjects as well as patients with inflammatory, dystrophic and denervating diseases were examined. By immunohistochemistry the main observations were (1) satellite cells expressed the non-sialylated form of NCAMs; (2) regenerative fibers strikingly expressed NCAMs and their sialylated isoforms both on membranes and in the cytoplasm; (3) in denervated muscles, fibers in atrophic groups and some fibers in acute denervation expressed NCAMs on their membrane but not the highly sialylated form; (4) finally, some fibers in myotonic dystrophy and fibers with rimmed vacuoles also expressed NCAMs. Biochemical approaches, using enzymes such as endoglycosidase N and phosphatidylinositol phospholipase C combined with immunoblot analysis allowed visualization of the nature of the expressed isoforms. We have shown that non activated cells, i.e. satellite cells and denervated fibers do not express polysialylated NCAMs. This post-translational modification may be only observed in activated or regenerating fibers. This would parallel the sequence of NCAM expression occurring in normal myogenic pathways.
针对神经细胞黏附分子（NCAM）的抗体，尤其是识别多唾液酸化异构体的单克隆抗体，被用于表征这些分子在正常和患病人类肌肉中的表达情况。研究对象包括正常受试者以及患有炎症性、营养不良性和失神经疾病的患者。通过免疫组织化学方法，主要观察结果如下：（1）卫星细胞表达非唾液酸化形式的NCAM；（2）再生纤维在细胞膜和细胞质中均显著表达NCAM及其唾液酸化异构体；（3）在失神经肌肉中，萎缩组的纤维以及急性失神经时的一些纤维在其细胞膜上表达NCAM，但不表达高度唾液酸化形式；（4）最后，强直性肌营养不良中的一些纤维以及有镶边空泡的纤维也表达NCAM。采用内切糖苷酶N和磷脂酰肌醇磷脂酶C等酶结合免疫印迹分析的生化方法，能够观察到所表达异构体的性质。我们已经表明，未活化的细胞，即卫星细胞和失神经纤维不表达多唾液酸化的NCAM。这种翻译后修饰可能仅在活化或再生的纤维中观察到。这与正常肌生成途径中NCAM表达的顺序相平行。
