Billings-Gagliardi S, Adcock L H, Schwing G B, Wolf M K
Brain Res. 1980 Oct 27;200(1):135-50. doi: 10.1016/0006-8993(80)91100-2.
Organotypic cultures of cerebellum from hypomyelinated mutant mice provide a powerful experimental system for studying the cell biology of the mutant diseases. We have examined the extent to which the culture system reproduces the diseases of three well-known mutants, qk, jpmsd, and jp. Quantitation of myelin profiles per sq. mm of section demonstrates that in vitro, as in situ, qk produces the most myelin jpmsd an intermediate amount, and jp the least. Myelin in qk cultures is unique in being invisible by light microscopy of the living culture. Hypomyelination of jp may be more severe in vitro than in situ. Cultures of jpmsd exhibit many of the ultrastructural features of cerebellar abnormalities that occur in situ: degree of hypomyelination, clustering of myelin segments, scarcity of oligodendrocytes, absence of nodes of Ranvier but presence of heminodes, and apparent structural integrity of the myelin sheaths. Correspondence between in vitro and in situ ultrastructure is more difficult to assess for jp, because the available sample of jp myelin in vitro is too small, and for qk, because the abnormalities observed in situ resemble nonspecific abnormalities of normal myelin in vitro.
来自髓鞘形成不足突变小鼠的小脑器官型培养物为研究突变疾病的细胞生物学提供了一个强大的实验系统。我们已经研究了该培养系统在多大程度上重现了三种著名突变体qk、jpmsd和jp的疾病情况。对每平方毫米切片的髓鞘轮廓进行定量分析表明，在体外，如同在原位一样，qk产生的髓鞘最多，jpmsd产生的量居中，而jp产生的最少。qk培养物中的髓鞘具有独特性，在活培养物的光学显微镜下不可见。jp的髓鞘形成不足在体外可能比在原位更严重。jpmsd的培养物展现出许多在原位出现的小脑异常的超微结构特征：髓鞘形成不足的程度、髓鞘节段的聚集、少突胶质细胞的稀少、郎飞结的缺失但有半结的存在，以及髓鞘鞘膜明显的结构完整性。对于jp，由于体外可用的jp髓鞘样本太小，而对于qk，由于在原位观察到的异常类似于体外正常髓鞘的非特异性异常，所以更难评估体外和原位超微结构之间的对应关系。
