New insights into the structural roles of nebulin in skeletal muscle.
New insights into the structural roles of nebulin in skeletal muscle.
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关于星云蛋白在骨骼肌中的结构作用的新见解。
DOI：
10.1155/2010/968139
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发表时间：
2010
影响因子：
--
通讯作者：
Granzier,Henk
中科院分区：
文献类型：
--
作者：
Ottenheijm,CoenAC;Granzier,Henk
肌肉结构和功能的一个重要特征仍然相对模糊，那就是调节细丝长度的机制。肌丝长度是肌肉功能的一个重要方面，因为产生的力与粗肌丝和细肌丝之间的重叠量成正比。最近的进展，部分归因于星云蛋白 KO 模型的产生，揭示了星云蛋白在细丝长度的调节中发挥着重要作用。骨骼肌的另一个尚未被充分理解的结构特征是维持相邻肌节的规则横向排列所涉及的机制，即肌原纤维连接性。最近的研究表明，星云蛋白是机械连接相邻肌原纤维的蛋白质复合物的一部分。因此，星云蛋白在骨骼肌中的新结构作用涉及细丝长度的调节和维持肌原纤维的连接。当星云蛋白的这些功能缺失时，就会出现肌肉无力，就像患有星云蛋白突变的线状肌病患者的情况一样。在这里，我们回顾了星云蛋白在骨骼肌结构中的作用的新见解。
One important feature of muscle structure and function that has remained relatively obscure is the mechanism that regulates thin filament length. Filament length is an important aspect of muscle function as force production is proportional to the amount of overlap between thick and thin filaments. Recent advances, due in part to the generation of nebulin KO models, reveal that nebulin plays an important role in the regulation of thin filament length. Another structural feature of skeletal muscle that is not well understood is the mechanism involved in maintaining the regular lateral alignment of adjacent sarcomeres, that is, myofibrillar connectivity. Recent studies indicate that nebulin is part of a protein complex that mechanically links adjacent myofibrils. Thus, novel structural roles of nebulin in skeletal muscle involve the regulation of thin filament length and maintaining myofibrillar connectivity. When these functions of nebulin are absent, muscle weakness ensues, as is the case in patients with nemaline myopathy with mutations in nebulin. Here we review these new insights in the role of nebulin in skeletal muscle structure.