characterization of the laminin-dystroglycan interaction in neuropathy and its therapeutic application

雪旺细胞中的肌营养不良聚糖与基底膜中的层粘连蛋白之间的相互作用对于周围神经的髓鞘形成至关重要。我们培育出大型转基因小鼠，其中这种相互作用大大增强。在这些小鼠的周围神经中，一些与髓鞘形成相关的基因的表达发生了改变。我们将大型转基因小鼠与 dy2J 小鼠杂交，后者表现出周围神经髓鞘形成障碍，但髓鞘形成缺陷并未得到改善。由于周围神经通过神经肌肉接头影响骨骼肌的再生，因此我们检查了 dy2J 小鼠骨骼肌的再生。我们证明，这些小鼠中 LARGE 的过度表达会抑制肌肉再生。

The interaction between dystroglycan in Schwann cell and laminin in the basement membrane is crucial for the myelination of peripheral nerve. We generated LARGE transgenic mice in which this interaction is greatly enhanced. In the peripheral nerves of these mice, the expression of some genes related to myelination was altered. We crossed LARGE transgenic mice with dy2J mice, which exhibit dysmyelination of the peripheral nerve, however the defects in myelination were not ameliorated. Since the peripheral nerve affects the regeneration of the skeletal muscle via neuromuscular junction, we examined the regeneration of dy2J mice skeletal muscles. We demonstrated that the muscle regeneration was suppressed by the overexpression of LARGE in these mice.