Emery-Dreifuss muscular dystrophy-linked genes and centronuclear myopathy-linked genes regulate myonuclear movement by distinct mechanisms.
Emery-Dreifuss muscular dystrophy-linked genes and centronuclear myopathy-linked genes regulate myonuclear movement by distinct mechanisms.
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DOI：
10.1091/mbc.e16-10-0721
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发表时间：
2017-08-15
影响因子：
3.3
通讯作者：
Folker ES
中科院分区：
文献类型：
--
作者：
Collins MA;Mandigo TR;Camuglia JM;Vazquez GA;Anderson AJ;Hudson CH;Hanron JL;Folker ES
果蝇被用作一个模型系统，以显示常见的表型错位核发生通过不同的机制在Emery-Dreifuss肌营养不良症和中枢性肌病。肌细胞是一个合胞体，其中许多核的位置使相邻核之间的距离最大化。虽然错位的核与许多肌肉疾病相关，但尚不清楚这种常见的表型是否是共同机制的结果。为了回答这个问题，我们破坏了果蝇中与Emery-Dreifuss肌营养不良症（EDMD）和中枢性肌病（CNM）相关的基因的表达，并评估了细胞核的位置。我们发现与EDMD和CNM相关的基因都是正确定位细胞核所必需的。然而，具体的表型不同。EDMD连锁基因是必要的细胞核的初始分离成不同的集群，这表明这些因素缓解细胞核之间的相互作用。CNM连锁基因是必要的，以维持核簇内，因为它们向肌肉末端移动，这表明这些因素是必要的，以维持核之间的相互作用。总之，这些数据表明，在EDMD和CNM中，核位置被不同的机制破坏。
Drosophila is used as a model system to show that the common phenotype of mispositioned nuclei occurs via distinct mechanisms in Emery–Dreifuss muscular dystrophy and centronuclear myopathy. Muscle cells are a syncytium in which the many nuclei are positioned to maximize the distance between adjacent nuclei. Although mispositioned nuclei are correlated with many muscle disorders, it is not known whether this common phenotype is the result of a common mechanism. To answer this question, we disrupted the expression of genes linked to Emery–Dreifuss muscular dystrophy (EDMD) and centronuclear myopathy (CNM) in Drosophila and evaluated the position of the nuclei. We found that the genes linked to EDMD and CNM were each necessary to properly position nuclei. However, the specific phenotypes were different. EDMD-linked genes were necessary for the initial separation of nuclei into distinct clusters, suggesting that these factors relieve interactions between nuclei. CNM-linked genes were necessary to maintain the nuclei within clusters as they moved toward the muscle ends, suggesting that these factors were necessary to maintain interactions between nuclei. Together these data suggest that nuclear position is disrupted by distinct mechanisms in EDMD and CNM.