Sensory nerve-dominant nerve degeneration and remodeling in the mutant mice lacking complex gangliosides
Sensory nerve-dominant nerve degeneration and remodeling in the mutant mice lacking complex gangliosides
复制标题
DOI：
10.1016/j.neuroscience.2005.07.035
复制
发表时间：
2005-01-01
期刊：
影响因子：
3.3
通讯作者：
Furukawa, K
中科院分区：
文献类型：
--
作者：
Sugiura, Y;Furukawa, K;Furukawa, K
神经节苷类，含唾液酸的鞘糖脂，在哺乳动物神经系统中丰富。由于产生了(31,4- n -乙酰半乳糖氨基转移酶（GM2/GD2合成酶）中断的突变小鼠，因此对突变小鼠的病理学进行了一些研究，即轻度功能障碍和周围神经系统的沃勒氏变性。为了进一步分析突变小鼠的时间变化，我们主要通过形态学方法（如电镜和免疫组织化学）检查外周和中枢神经系统。随着感觉功能障碍的出现，成年小鼠脊髓背角出现神经退行性变、胶质细胞增生和突触重构。厚的星形胶质细胞突起在神经丸之间和血管周围延伸，胶质丝密集堆积。突触囊泡的形态变化和突触与中枢末梢的接触方式显示突触在变性后发生了重塑。这些结果表明，复杂神经节苷在维持神经系统结构和功能的完整性方面是必不可少的，缺乏神经节苷会导致以感觉神经为主的神经变性。(c) 2005 IBRO。Elsevier Ltd.出版。版权所有。
Gangliosides, sialic acid-containing glycosphingolipids, are enriched in the mammalian nervous system. Since mutant mice with disrupted (31,4-N-acetylgalactosaminyltransferase (GM2/GD2 synthase) were generated, there have been several studies on the pathology of the mutant mice, i.e. mild functional disorders and Wallerian degeneration in the peripheral nervous system. To further analyze the chronological alteration in the mutant mice, we examined the peripheral and CNS mainly with morphological approaches, such as electron microscopy and immunohistochemistry. Accordingly with the sensory dysfunction, neural degeneration, glial proliferation and synaptic remodeling in the dorsal horn of the spinal cord were found in adult mice. Thick astrocytic processes with densely packed glial filaments were extended among the neuropils and around blood vessels. Morphological changes in the synaptic vesicles and modes of synaptic contacts with central terminals were detected, suggesting synaptic remodeling following the degeneration. These results suggest that complex gangliosides are essential in the maintenance of integrity in architecture and function of the nervous system, lack of which results in neural degeneration in a sensory nerve-dominant manner. (c) 2005 IBRO. Published by Elsevier Ltd. All rights reserved.