PDF(Pubmed)
Abstract：
Dystroglycan is a ubiquitously expressed heterodimeric cell-surface laminin receptor with roles in cell adhesion, signalling, and membrane stabilisation. More recently, the transmembrane β-subunit of dystroglycan has been shown to localise to both the nuclear envelope and the nucleoplasm. This has led to the hypothesis that dystroglycan may have a structural role at the nuclear envelope analogous to its role at the plasma membrane. The biochemical fraction of myoblast cells clearly supports the presence of dystroglycan in the nucleus. Deletion of the dystroglycan protein by disruption of the DAG1 locus using CRISPR/Cas9 leads to changes in nuclear size but not overall morphology; moreover, the Young\'s modulus of dystroglycan-deleted nuclei, as determined by atomic force microscopy, is unaltered. Dystroglycan-disrupted myoblasts are also no more susceptible to nuclear stresses including chemical and mechanical, than normal myoblasts. Re-expression of dystroglycan in DAG1-disrupted myoblasts restores nuclear size without affecting other nuclear parameters.
摘要：
Dystroglycan是一种广泛表达的异二聚体细胞表面层粘连蛋白受体，在细胞粘附中起作用，信令,和膜稳定。最近,已显示，肌营养不良聚糖的跨膜β亚基定位于核膜和核质。这导致了这样的假设，即营养不良聚糖可能在核膜上具有类似于其在质膜上的作用的结构作用。成肌细胞的生化部分清楚地支持在细胞核中存在营养不良聚糖。通过使用CRISPR/Cas9破坏DAG1基因座来缺失营养不良蛋白聚糖蛋白会导致细胞核大小的变化，但不会导致整体形态的变化；此外，营养不良聚糖缺失细胞核的杨氏模量，由原子力显微镜测定，是不变的。营养不良聚糖破坏的成肌细胞也不再容易受到核应激，包括化学和机械应激，比正常成肌细胞.在DAG1破坏的成肌细胞中，肌营养不良蛋白聚糖的再表达可恢复核大小，而不影响其他核参数。
