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1、SICENCE 新闻发布 作者: Wensheng Lin 稿号：NRR-D-14-00400真核翻译起始因子真核翻译起始因子2B细胞：会自主引起发育过程中髓鞘少突胶质细胞功能障碍细胞：会自主引起发育过程中髓鞘少突胶质细胞功能障碍消融性白质脑病，也被称为儿童共济失调和中枢神经系统低髓鞘形成，是一种遗传性常 染色体隐性染性脑白质营养不良疾病。消融性白质脑病的病理特征，如小脑性共济失调 和痉挛通常始于婴儿期后期或幼年早期。消融性白质脑病是慢性渐进性疾病，感染性发 热或轻微头部外伤会引起病症的迅速恶化。少突胶质细胞主要影响消融性白质脑病，并 表现出特有的泡沫形态。虽然少突胶质细胞数量在严重损伤白质中
2、会有减少，但在损伤 较小区域会有所增加。此外，最近的一项研究表明，消融性白质脑病的病理特征会抑制 少突胶质细胞的髓鞘形成功能。近日，Wensheng Lin 教授在发表于中国神经再生研 究（英文版） 杂志（2015 年 10 卷 2 期）的观点文章中提出，真核翻译起始因子 2B（eIF2B）突变被认为是消融性白质脑病患者丧失功能的主要原因。而少突胶质细胞 对蛋白质转译干扰非常敏感。因此，有一种可能性，即 eIF2B 突变对蛋白质生物合成的 抑制作用会首先引起少突胶质细胞功能障碍，并有助于消融性白质脑病的发病机制。他 们最近的研究表明使用强大基因工具可以选择性地损伤少突胶质细胞中的 eIF2B
3、活动， eIF2B 细胞会自主引起发育过程中髓鞘少突胶质细胞功能障碍，导致消融性白质脑病的 病理损害。然而，由于小鼠模型系统有其局限性，进一步的研究是必要的，用以证实少 突胶质细胞的 eIF2B 障碍在消融性白质脑病发病机制中的细胞自主角色。Article: “Impaired eIF2B activity in ligodendrocytes contributes to VWMD pathogenesis“ by Wensheng Lin (Department of Neuroscience, University of Minnesota, Minneapolis, MN, USA;
4、Institute for Translational Neuroscience, University of Minnesota, Minneapolis, MN, USA)Lin W (2015) Impaired eIF2B activity in oligodendrocytes contributes to VWMD pathogenesis. Neural Regen Res 10(2):195-197.欲获更多资讯：Neural Regen ResSICENCE 新闻发布 作者: Wensheng Lin 稿号：NRR-D-14-00400Impaired eIF2B activ
5、ity in myelinating oligodendrocytes dysfunction contributes to VWMD pathogenesisVanishing white matter disease (VWMD, MIM #603896), also known as Childhood Ataxia with CNS Hypomyelination (CACH), is an inherited autosomal-recessive leukodystrophy. The neurological signs of VWMD, such as cerebellar a
6、taxia and spasticity, usually begin in late infancy or early childhood. VWMD is chronic and progressive, with additional episodes of rapid deterioration after febrile infection or minor head trauma. Oligodendrocytes are predominantly affected in VWMD, which exhibit characteristic foamy morphology. A
7、lthough oligodendrocyte numbers are decreased in the severely affected white matter, the numbers are increased in the less affected areas. Additionally, a recent study suggests that the myelinating function of oligodendrocytes is suppressed in VWMD patients. Recently, Prof. Wensheng Lin (University
8、of Minnesota, USA) published a perspective article in Neural Regeneration Research (Vol. 10, No. 2, 2015), they considered mutations in the ubiquitously-expressed genes encoding the five subunits of eukaryotic translation initiation factor 2B (eIF2B) are believed to be the cause of VWMD. Not surpris
9、ingly, oligodendrocytes are highly sensitive to the disruption of protein translation. Therefore, there is a possibility that the inhibitory effects of eIF2B mutations on protein biosynthesis preferentially cause oligodendrocyte dysfunction and contribute to the pathogenesis of VWMD. Using a powerfu
10、l genetic tool for selectively impairing eIF2B activity in oligodendrocytes, our recent study indicates that impairment of eIF2B cell-autonomously causes myelinating oligodendrocyte dysfunction during developmental myelination, resulting in VWMD pathology. Nevertheless, their mouse model system has
11、its limits; additional studies are essential and necessary to verify the cell autonomous role of eIF2B impairment in oligodendrocytes in VWMD pathogenesis.Article: “Impaired eIF2B activity in ligodendrocytes contributes to VWMD pathogenesis“ by Wensheng Lin (Department of Neuroscience, University of Minnesota, Minneapolis, MN, USA; Institute for Translational Neuroscience, University of Minnesota, Minneapolis, MN, USA)Lin W (2015) Impaired eIF2B activity in oligodendrocytes contributes to VWMD pathogenesis. Neural Regen Res 10(2):195-197.
