Huard J, Labrecque C, Dansereau G, Robitaille L, Tremblay J P
Laboratoire de Neurobiologie, Université Laval, Hôpital de l'Enfant-Jesus, Québec, Canada.
Muscle Nerve. 1991 Feb;14(2):178-82. doi: 10.1002/mus.880140213.
Mdx mouse dystrophy is characterized by the absence in the muscle cytoplasmic membrane of a high molecular weight protein called dystrophin. A possible avenue for treatment of muscular dystrophies is to inject normal myoblasts in a dystrophic muscle to form hybrid muscle fibers. Hybrid myotubes were formed in vitro by the fusion of normal rat and dystrophic mouse (mdx) myoblasts. Staining with Hoechst dye 33258 permitted the clear distinction of mouse and rat nuclei. Immunostaining demonstrated that dystrophin was present over the entire membrane of all hybrid myotubes even when nuclei ratio normal/dystrophic was low.
mdx小鼠肌营养不良的特征是，在肌肉细胞质膜中缺乏一种名为抗肌萎缩蛋白的高分子量蛋白质。治疗肌肉萎缩症的一种可能途径是，向营养不良的肌肉中注射正常的成肌细胞，以形成混合肌纤维。正常大鼠成肌细胞和营养不良小鼠（mdx）成肌细胞在体外融合，形成了混合肌管。用Hoechst 33258染料染色，可以清晰区分小鼠和大鼠的细胞核。免疫染色表明，即使正常/营养不良细胞核的比例很低，所有混合肌管的整个膜上都存在抗肌萎缩蛋白。
