Samitt C E, Bonilla E
Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York 10032.
Muscle Nerve. 1990 Jun;13(6):493-500. doi: 10.1002/mus.880130605.
Dystrophin is the protein whose deficiency results in Duchenne muscular dystrophy. The protein has homologies with a number of cytoskeletal proteins, is localized at the muscle sarcolemma and it may provide stability to the muscle plasma membrane. Using immunocytochemical techniques, we have studied dystrophin localization at the myotendinous junction, a region of membrane complexity that requires more stability because it is subjected to great mechanical stress during the transmission of contractile force to the tendon. The results showed subsarcolemmal deposits of dystrophin at the junctional folds of the myotendon as well as membrane-associated dystrophin at extrajunctional sarcolemma. The findings suggest that dystrophin may be one of the components linking terminal actin filaments to the subplasmalemmal surface of the junctional folds of the myotendon.
肌营养不良蛋白是一种蛋白质,其缺乏会导致杜氏肌营养不良症。该蛋白与多种细胞骨架蛋白具有同源性,定位于肌肉肌膜,可能为肌肉质膜提供稳定性。我们使用免疫细胞化学技术研究了肌营养不良蛋白在肌腱结合部的定位,肌腱结合部是一个膜结构复杂的区域,由于在收缩力传递到肌腱的过程中会受到很大的机械应力,因此需要更高的稳定性。结果显示,在肌腱结合部的连接褶皱处有肌营养不良蛋白的肌膜下沉积,以及在结合部外肌膜处有与膜相关的肌营养不良蛋白。这些发现表明,肌营养不良蛋白可能是将终末肌动蛋白丝与肌腱结合部连接褶皱的质膜下表面相连的成分之一。