Wakayama Yoshihiro
Department of Neurology, Showa University Fujigaoka Hospital, Aoba-ku, Yokohama, Japan.
J Biomed Biotechnol. 2010;2010:731569. doi: 10.1155/2010/731569. Epub 2010 Mar 21.
Freeze-fracture electron microscopy enabled us to observe the molecular architecture of the biological membranes. We were studying the myofiber plasma membranes of health and disease by using this technique and were interested in the special assembly called orthogonal arrays (OAs). OAs were present in normal myofiber plasma membranes and were especially numerous in fast twitch type 2 myofibers; while OAs were lost from sarcolemmal plasma membranes of severely affected muscles with dystrophinopathy and dysferlinopathy but not with caveolinopathy. In the mid nineties of the last century, the OAs turned out to be a water channel named aquaporin 4 (AQP4). Since this discovery, several groups of investigators have been studying AQP4 expression in diseased muscles. This review summarizes the papers which describe the expression of OAs, AQP4, and other AQPs at the sarcolemma of healthy and diseased muscle and discusses the possible role of AQPs, especially that of AQP4, in normal and pathological skeletal muscles.
冷冻蚀刻电子显微镜使我们能够观察生物膜的分子结构。我们正在使用这项技术研究健康和患病状态下的肌纤维质膜，并对一种名为正交阵列（OAs）的特殊结构感兴趣。OAs存在于正常肌纤维质膜中，在快肌纤维2型中尤其丰富；而在患有肌营养不良症和dysferlinopathy的严重受影响肌肉的肌膜质膜中OAs消失，但在患有小窝蛋白病的肌肉中则没有。在上个世纪九十年代中期，OAs被证明是一种名为水通道蛋白4（AQP4）的水通道。自这一发现以来，几组研究人员一直在研究AQP4在患病肌肉中的表达。这篇综述总结了描述健康和患病肌肉肌膜上OAs、AQP4和其他水通道蛋白表达的论文，并讨论了水通道蛋白，尤其是AQP4，在正常和病理性骨骼肌中的可能作用。
