Pons F, Augier N, Léger J O, Robert A, Tomé F M, Fardeau M, Voit T, Nicholson L V, Mornet D, Léger J J
Pathologie Générale, Faculté de Médecine, Montpellier, France.
FEBS Lett. 1991 Apr 22;282(1):161-5. doi: 10.1016/0014-5793(91)80468-i.
Polyclonal and monoclonal antibodies, which recognize different regions and epitopes of the dystrophin molecule, bind to a protein of Mr 400,000 which is present in extracts of mdx muscle from regions which contain neuromuscular junctions (NMJ) and is absent from those which do not. This NMJ-associated homologue of dystrophin has at least 2 epitopes which are different to usual Xp21 form of dystrophin expressed along the sarcolemma of muscle fibres in normal muscles. This protein is also expressed at the NMJ of a DMD patient who lacks the first 52 exons of the Xp21 dystrophin gene and it must therefore be translated from a different gene transcript.
多克隆抗体和单克隆抗体可识别抗肌萎缩蛋白分子的不同区域和表位，它们与一种分子量为400,000的蛋白质结合，该蛋白质存在于含有神经肌肉接头（NMJ）区域的mdx肌肉提取物中，而在不含有该区域的提取物中则不存在。这种与神经肌肉接头相关的抗肌萎缩蛋白同系物至少有2个表位，与正常肌肉中沿肌纤维肌膜表达的常见Xp21形式的抗肌萎缩蛋白不同。这种蛋白质也在一名缺乏Xp21抗肌萎缩蛋白基因前52个外显子的杜氏肌营养不良症（DMD）患者的神经肌肉接头上表达，因此它一定是从不同的基因转录本翻译而来的。
