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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Disruption of axo-glial junctions causes cytoskeletal disorganization and degeneration of Purkinje neuron axons
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Disruption of axo-glial junctions causes cytoskeletal disorganization and degeneration of Purkinje neuron axons

机译：轴突-胶质连接的破坏引起浦肯野神经元轴突的细胞骨架紊乱和变性

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摘要

Axo-glial junctions (AGJs) play a critical role in the organization and maintenance of molecular domains in myelinated axons. Neurexin IV/Caspr1/paranodin (NCP1) is an important player in the formation of AGJs because it recruits a paranodal complex implicated in the tethering of glial proteins to the axonal membrane and cytoskeleton. Mice deficient in either the axonal protein NCP1 or the glial ceramide galactosyltransferase (CGT) display disruptions in AGJs and severe ataxia. in this article, we correlate these two phenotypes and show that both NCP1 and CGT mutants develop large swellings accompanied by cytoskeletal disorganization and degeneration in the axons of cerebellar Purkinje neurons. We also show that all spectrin is part of the paranodal complex and that, although not properly targeted, this complex is still formed in CGT mutants. Together, these findings establish a physiologically relevant link between AGJs and axonal cytoskeleton and raise the possibility that some neurodegenerative disorders arise from disruption of the AGJs.
机译：轴突-神经胶质连接（AGJs）在有髓轴突的分子域的组织和维持中起关键作用。 Neurexin IV / Caspr1 / paranodin（NCP1）是AGJ形成的重要参与者，因为它募集了一种牵涉到神经胶质蛋白束缚到轴突膜和细胞骨架中的寄生性复合物。轴突蛋白NCP1或神经胶质神经酰胺半乳糖基转移酶（CGT）缺乏的小鼠在AGJs和严重的共济失调中表现出破坏。在本文中，我们将这两个表型相关联，并表明NCP1和CGT突变体均会在小脑浦肯野神经元轴突中发生大肿胀，并伴有细胞骨架紊乱和变性。我们还显示，所有血影蛋白都是旁淋巴结复合物的一部分，尽管未正确靶向，但该复合物仍在CGT突变体中形成。总之，这些发现在AGJ与轴突细胞骨架之间建立了生理相关的联系，并增加了某些神经退行性疾病由AGJ的破坏引起的可能性。

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