Rivier F, Robert A, Latouche J, Hugon G, Mornet D
Pathologie Moléculaire du Muscle, INSERM U. 300, Faculté de Pharmacie, Montpellier, France.
Comp Biochem Physiol B Biochem Mol Biol. 1997 Jan;116(1):19-26. doi: 10.1016/s0305-0491(96)00198-8.
By comparison with localizations of dystrophin family products in rabbit peripheral nerves, we investigated the potential existence and distribution of similar products in peripheral nerves from Torpedo marmorata. In immunofluorescence studies, a specific set of monoclonal antibodies directed against dystrophin family proteins clearly stained a thin rim surrounding each Schwann cell-axon unit both in T. marmorata and rabbit peripheral nerves. In contrast when using the dystrophin/utrophin monoclonal H'3E7 antibody, we found a clear difference between rabbit and T. marmorata peripheral nerves according to fluorescent labeling detected within Torpedo nerve axons. Further differences were noted following western blot analyses of T. marmorata peripheral nerve extracts, highlighting the presence of a new and specific M(r) 70-kDa protein band belonging to the dystrophin family, which is localized within axons in addition to: (1) an M(r)400-kDa protein band detected with dystrophin/utrophin antibodies; and (2) an M(r) 116-kDa doublet protein band corresponding to Dp116 and Up116 isoforms. All of these products, detected according to the specificities of the monoclonal antibodies used, are discussed in terms of their potential identities as short and long dystrophin or utrophin mammalian products.
通过比较肌营养不良蛋白家族产物在兔外周神经中的定位,我们研究了电鳐外周神经中类似产物的潜在存在和分布情况。在免疫荧光研究中,一组针对肌营养不良蛋白家族蛋白的特异性单克隆抗体,在电鳐和兔外周神经中均清晰地染出围绕每个雪旺细胞 - 轴突单元的薄边缘。相比之下,当使用肌营养不良蛋白/抗肌萎缩蛋白单克隆抗体H'3E7时,根据在电鳐神经轴突内检测到的荧光标记,我们发现兔和电鳐外周神经之间存在明显差异。在对电鳐外周神经提取物进行蛋白质印迹分析后,还发现了进一步的差异,突出显示了属于肌营养不良蛋白家族的一种新的特异性M(r) 70-kDa蛋白条带的存在,该条带除了定位于轴突内之外,还包括:(1) 用肌营养不良蛋白/抗肌萎缩蛋白抗体检测到的M(r) 400-kDa蛋白条带;以及 (2) 对应于Dp116和Up116亚型的M(r) 116-kDa双峰蛋白条带。根据所用单克隆抗体的特异性检测到的所有这些产物,就其作为短和长肌营养不良蛋白或抗肌萎缩蛋白哺乳动物产物的潜在身份进行了讨论。