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首页> 外文期刊>Zoological Science >Chimeric Mice with Deletion of Cfl2 that Encodes Muscle-Type Cofilin (MCF or Cofilin-2) Results in Defects of Striated Muscles, Both Skeletal and Cardiac Muscles
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Chimeric Mice with Deletion of Cfl2 that Encodes Muscle-Type Cofilin (MCF or Cofilin-2) Results in Defects of Striated Muscles, Both Skeletal and Cardiac Muscles

机译：嵌入CFL2的嵌合小鼠，编码肌肉型Cofilin（MCF或Cofilin-2）导致骨骼和心肌缺陷的椎体缺陷

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摘要

Cofilin, a member of the ADF/cofilin family, is an actin-binding protein which is widely distributed among eukaryotic organisms and involved in actin filament dynamics in a variety of cell types. In mammalian striated muscles, muscle-type cofilin (MCF or cofilin-2) is predominantly expressed. Previous investigations have shown that MCF plays an essential role in the regulation of assembly of contractile apparatus in skeletal muscle, but its role in cardiac muscle has remained unclear. In the present study, in order to further clarify the role of MCF in organization of myofibrillar structure in vivo, we generated chimeric mice with a combination of MCF-deficient cells that were generated by Cfl2-knockout (Cfl2(-)(/-)) and wild type cells containing MCF, and examined the effect of MCF deficiency on striated muscles, especially on the fine structures of contractile apparatus in cardiac muscle. We found that mice chimeric for MCF deficient cells exhibited structural defects in their skeletal muscles as previously reported. Histological analysis showed that MCF deficiency leads to degradation of myofibers and promotion of muscle regeneration. Electron microscopic observation of cardiac muscle of the chimeric mice showed coexistence of the cells with normal sarcomeres and those with disorganized myofibrils in a chimeric pattern. In these cofilin-deficient cells, myofilaments were scattered in the cytoplasm and myofibrillar structures were severely disrupted. These results provide strong evidence for that MCF plays a critical role in the formation and the maintenance of myofibril structure not only in skeletal muscle but also in cardiac muscle.
机译：ADF / Cofilin家族的成员是一种肌动蛋白结合蛋白，其广泛分布在真核生物中，并参与各种细胞类型中的肌动蛋白灯丝动力学。在哺乳动物条纹的肌肉中，主要表达肌肉型Cofilin（MCF或Cofilin-2）。以前的调查表明，MCF在骨骼肌中收缩仪器组装的调节中起重要作用，但其在心肌中的作用仍然不清楚。在本研究中，为了进一步阐明MCF在体内组织Myofibrillar结构中的作用，我们将嵌合小鼠产生了由CFL2敲除产生的MCF缺陷细胞组合（CFL2（ - ）（/ - ） ）含有MCF的野生型细胞，并检查了MCF缺乏对条纹肌肉的影响，特别是在心肌中收缩装置的细结构。我们发现，如前所述，用于MCF缺陷细胞的小鼠嵌合缺陷细胞在其骨骼肌中表现出结构缺陷。组织学分析表明，MCF缺乏导致肌纤维的降解和促进肌肉再生。电子显微镜观察嵌合小鼠的心肌肌肉显示细胞与正常肉体的共存，嵌合图案中具有杂皮灭菌的细胞的共存。在这些辛菌素缺陷的细胞中，肌丝散落在细胞质中，并且肌原纤维结构严重破坏。这些结果为MCF提供了强有力的证据，即MCF在形成和维持肌纤维结构中不仅在骨骼肌中的维持，而且在心肌中的肌肉。

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