Collins Mary Ann, Mandigo Torrey R, Camuglia Jaclyn M, Vazquez Gabriella A, Anderson Alyssa J, Hudson Christine H, Hanron John L, Folker Eric S
Biology Department, Boston College, Chestnut Hill, MA 02467.
Biology Department, Boston College, Chestnut Hill, MA 02467
Mol Biol Cell. 2017 Aug 15;28(17):2303-2317. doi: 10.1091/mbc.E16-10-0721. Epub 2017 Jun 21.
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 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.
肌细胞是一种多核体，其中许多细胞核的定位方式能使相邻细胞核之间的距离最大化。尽管细胞核位置异常与多种肌肉疾病相关，但尚不清楚这种常见表型是否由共同机制导致。为回答这个问题，我们在[具体内容缺失]中破坏了与埃默里-德赖富斯肌营养不良症（EDMD）和中央核肌病（CNM）相关的基因表达，并评估了细胞核的位置。我们发现，与EDMD和CNM相关的基因对于细胞核的正确定位都是必需的。然而，具体表型有所不同。与EDMD相关的基因对于细胞核最初分离成不同簇是必需的，这表明这些因素减轻了细胞核之间的相互作用。与CNM相关的基因在细胞核向肌端移动时维持其在簇内是必需的，这表明这些因素对于维持细胞核之间的相互作用是必需的。这些数据共同表明，在EDMD和CNM中，细胞核位置是由不同机制破坏的。
