Tolbert D L, Ewald M, Gutting J, La Regina M C
Francis and Doris Murphy Neuroanatomy Research Laboratory, Department of Anatomy and Neurobiology, St. Louis University School of Medicine, Missouri 63104, USA.
J Comp Neurol. 1995 May 15;355(4):490-507. doi: 10.1002/cne.903550403.
Temporal-spatial patterns of surviving Purkinje cells were studied quantitatively in a rat mutant (shaker) with differential hereditary cerebellar ataxia and Purkinje cell degeneration. Shaker rat mutants are characterized behaviorally as mild if they are ataxic or as strong if they have ataxia and tremor. Purkinje cells degenerate in both mild and strong shaker mutants, but the temporal and spatial patterns of cell death are strikingly different. In mild shaker mutants, Purkinje cell death is temporally restricted, with 31-46% of the Purkinje cells in lobules I-IX dying by 3 months of age. Very few Purkinje cells degenerate after this age. Purkinje cell death is spatially random. In lobules I-IX, every second, third, or fourth Purkinje cell degenerates. Purkinje cells in lobule X do not degenerate. In strong shaker mutants, Purkinje cell degeneration is temporally protracted and spatially restricted. By 3 months of age, most Purkinje cells in lobules I-VIa, -b, and -d have degenerated. Numerous Purkinje cells in the paravermis of lobules VIIb-VIII have also degenerated. Surviving Purkinje cells in the vermis and lateral hemisphere of lobules VIIb-VIII are aligned in parasagittally oriented stripes or transversely oriented bands. Purkinje cells continue to degenerate in localized areas of the posterior lobe such that, by 18 months of age, surviving Purkinje cells are limited primarily to lobules VIc, VIIa, IXd, and X. Quantitative analysis indicates that none of the Purkinje cells in these lobules degenerate.
在一种患有差异性遗传性小脑共济失调和浦肯野细胞变性的大鼠突变体（摇晃鼠）中，对存活的浦肯野细胞的时空模式进行了定量研究。摇晃鼠突变体在行为上的特征是，如果共济失调则为轻度，如果既有共济失调又有震颤则为重度。轻度和重度摇晃鼠突变体中的浦肯野细胞都会发生变性，但细胞死亡的时空模式却显著不同。在轻度摇晃鼠突变体中，浦肯野细胞死亡在时间上受到限制，到3月龄时，小叶I - IX中31% - 46%的浦肯野细胞死亡。在此年龄之后，很少有浦肯野细胞发生变性。浦肯野细胞死亡在空间上是随机的。在小叶I - IX中，每隔第二、第三或第四个浦肯野细胞发生变性。小叶X中的浦肯野细胞不会发生变性。在重度摇晃鼠突变体中，浦肯野细胞变性在时间上是持久的，在空间上是受限的。到3月龄时，小叶I - VIa、-b和-d中的大多数浦肯野细胞已经变性。小叶VIIb - VIII蚓旁的许多浦肯野细胞也已经变性。小叶VIIb - VIII蚓部和外侧半球中存活的浦肯野细胞以矢状旁向条纹或横向带的形式排列。浦肯野细胞在后叶的局部区域继续发生变性，以至于到18月龄时，存活的浦肯野细胞主要局限于小叶VIc、VIIa、IXd和X。定量分析表明，这些小叶中的浦肯野细胞均未发生变性。
