Shen X Y, Billings-Gagliardi S, Sidman R L, Wolf M K
Brain Res. 1985 Dec 23;360(1-2):235-47. doi: 10.1016/0006-8993(85)91239-9.
A new B6C3 stock of shimld mutant mice is compared in terms of behavior and CNS morphology with both a B6C3 shi stock and reports on other shimld animals. Defects of B6C3 shimld myelination seen at postnatal day 21 (P-21) are comparable to those in B6C3 shi with respect to % axons myelinated, sheath thickness, errors in the wrapping and targeting of myelin and abnormal oligodendrocyte shape. The two mutations are similarly expressed in cerebellar organotypic cultures. However, the major dense line (MDL) is present in a few shimld myelin sheaths at P-21 and a few sheaths show myelin basic protein by immunocytochemistry, while neither phenomenon is seen in shi at this age in the same CNS regions. Shimld mice survive their disease significantly better than shi. The shimld stock currently under study elsewhere differs from this B6C3 stock in that MDL was reported only in older animals, and behavior and survival were severely compromised.
将新的B6C3品系的shimld突变小鼠在行为和中枢神经系统形态方面与B6C3 shi品系以及其他shimld动物的报告进行了比较。在出生后第21天（P-21）观察到的B6C3 shimld髓鞘形成缺陷在髓鞘化轴突百分比、鞘厚度、髓鞘包裹和靶向错误以及少突胶质细胞形状异常方面与B6C3 shi中的缺陷相当。这两种突变在小脑器官型培养物中的表达相似。然而，在P-21时，少数shimld髓鞘中有主致密线（MDL），少数鞘通过免疫细胞化学显示有髓鞘碱性蛋白，而在相同中枢神经系统区域的这个年龄的shi中均未观察到这两种现象。Shimld小鼠的疾病存活率明显高于shi。目前在其他地方研究的shimld品系与这个B6C3品系的不同之处在于，仅在较年长的动物中报告有MDL，并且行为和存活率严重受损。
