Kondo A, Hoogerbrugge P M, Suzuki K, Poorthuis B J, Van Bekkum D W, Suzuki K
Department of Pathology, School of Medicine, University of North Carolina, Chapel Hill 27599.
Brain Res. 1988 Sep 13;460(1):178-83. doi: 10.1016/0006-8993(88)91220-6.
The peripheral nerve of the homozygous twitcher mouse (twi/twi), a murine model of globoid cell leukodystrophy (GLD), was examined following bone marrow transplantation (BMT). The light and electron microscopic studies revealed markedly increased numbers of remyelinated fibers and almost complete disappearance of the typical inclusion-laden macrophages in the trigeminal and sciatic nerves of the twi/twi which survived beyond 100 days of age. The pattern of remyelination appeared to be normal. GLD inclusions were still observed in the cytoplasm of some of the remyelinating Schwann cells and demyelinated fibers were still present in 108-day-old twitcher although no features of active demyelinating processes were observed. Thus, basic metabolic abnormality is still present despite clinical improvement in the twi/twi mouse following BMT.
对纯合子震颤小鼠（twi/twi）的周围神经进行了检查，该小鼠是球状细胞脑白质营养不良（GLD）的小鼠模型，检查在骨髓移植（BMT）之后进行。光镜和电镜研究显示，存活超过100日龄的twi/twi小鼠的三叉神经和坐骨神经中，重新髓鞘化的纤维数量显著增加，典型的充满包涵体的巨噬细胞几乎完全消失。重新髓鞘化的模式似乎正常。在一些重新髓鞘化的施万细胞的细胞质中仍观察到GLD包涵体，并且在108日龄的震颤小鼠中仍存在脱髓鞘纤维，尽管未观察到活跃脱髓鞘过程的特征。因此，尽管BMT后twi/twi小鼠临床症状有所改善，但基本代谢异常仍然存在。
