Takahashi H, Suzuki K
Acta Neuropathol. 1984;62(4):298-308. doi: 10.1007/BF00687612.
Chronologic events of demyelination were investigated in the spinal cord of the twitcher mouse, an authentic murine model of human globoid cell leukodystrophy (GLD) from 5 to 45 days postnatal. There was very little evidence of myelin degeneration before day 25 although clustered or scattered globoid cells were already noted in the dorsal columns and intramedullary portion of the ventral roots. Globoid cells contained typical cytoplasmic inclusions and in those which were found adjacent to degenerating myelin and naked axons, myelin debris were conspicuous in their cytoplasm. Vesiculation of myelin and a feature of globoid cells stripping myelin lamellae were noted in the area of demyelination. Myelin and oligodendroglial degeneration became pronounced throughout the spinal white matter after day 40 but globoid cells tended to be more concentrated in the dorsal columns. Our observations suggest that the emergence of globoid cells in GLD is in response to the changes in biochemical environment (i.e., excessive presence of galactosylceramide in the tissue?), and these cells appear to have a role as phagocytic cells in removing myelin lamellae.
在出生后5至45天的抽搐小鼠脊髓中研究了脱髓鞘的时间顺序事件。抽搐小鼠是人类球状细胞脑白质营养不良（GLD）的真实小鼠模型。在第25天之前，几乎没有髓鞘变性的证据，尽管在背柱和腹根的髓内部分已经发现了成簇或散在的球状细胞。球状细胞含有典型的细胞质内含物，在那些与变性髓鞘和裸露轴突相邻的球状细胞中，髓鞘碎片在其细胞质中很明显。在脱髓鞘区域注意到髓鞘的小泡形成以及球状细胞剥脱髓鞘板层的特征。在第40天之后，整个脊髓白质中的髓鞘和少突胶质细胞变性变得明显，但球状细胞倾向于更集中在背柱中。我们的观察结果表明，GLD中球状细胞的出现是对生化环境变化（即组织中半乳糖神经酰胺的过量存在？）的反应，并且这些细胞似乎具有作为吞噬细胞清除髓鞘板层的作用。
