Irino M, Suzuki K
Department of Pathology, School of Medicine, University of North Carolina, Chapel Hill.
Lab Invest. 1990 Jun;62(6):704-12.
Schwann cells were isolated and cultured from the twitcher mouse, an authentic model of human globoid cell leukodystrophy. We observed some difference in morphology and initial cell growth between the cells from suckling and adult mice. Initial growth was slower in homozygous and heterozygous twitcher (twi/twi; twi/+) cells than in normal cells (+/+) when cells were cultured from suckling mice. On the other hand, cell growth was initially accelerated in cells from adult twi/twi mice compared with adult twi/+ or +/+ animals. The yield of Schwann cells was much less from adults. In the cells from adult twi/twi, the perikaryon was swollen, and typical inclusions were easily recognized ultrastructurally, in particular after 14 days in vitro. However, only a few globoid cell leukodystrophy inclusions were detected in the cells from suckling twi/twi mice even after 28 days in vitro. The results indicate that Schwann cell pathology can be studied in twi/twi using an in vitro system and that twi/twi Schwann cells from suckling and adult mice have some differences in morphology as well as in the degree of initial growth.
从震颤小鼠（一种人类球形细胞脑白质营养不良的真实模型）中分离并培养施万细胞。我们观察到来自乳鼠和成年小鼠的细胞在形态和初始细胞生长方面存在一些差异。当从乳鼠中培养细胞时，纯合和杂合震颤小鼠（twi/twi；twi/+）细胞的初始生长比正常细胞（+/+）慢。另一方面，与成年twi/+或+/+动物相比，成年twi/twi小鼠的细胞初始生长加速。成年小鼠的施万细胞产量要少得多。在成年twi/twi的细胞中，核周体肿胀，在超微结构下很容易识别典型的包涵体，特别是在体外培养14天后。然而，即使在体外培养28天后，在乳鼠twi/twi的细胞中也仅检测到少数球形细胞脑白质营养不良包涵体。结果表明，可以使用体外系统在twi/twi中研究施万细胞病理学，并且来自乳鼠和成年小鼠的twi/twi施万细胞在形态以及初始生长程度上存在一些差异。
