Wüllner U, Weller M, Schulz J B, Krajewski S, Reed J C, Klockgether T
Department of Neurology, Eberhard-Karls University, Tübingen, Germany.
Acta Neuropathol. 1998 Sep;96(3):233-8. doi: 10.1007/s004010050889.
We investigated the expression of the apoptosis modulating proteins Bcl-2, Bax and Bcl-x in the cerebellum of mutant lurcher and weaver mice. Lurcher Purkinje cells and weaver germinal (granule neuron progenitor) cells both die via apoptosis during the postnatal cerebellar development. No significant changes in the expression patterns were detected prior to the actual cell death process. Instead apoptotic lurcher Purkinje cells exhibited increased Bax and Bcl-x expression, while surviving cells had an expression pattern similar to that of healthy littermates. Increased Bax expression was also found in apoptotic weaver germinal cells, while no change of Bcl-x expression was detected. Bcl-2 was expressed at low levels in cerebellar neurons and no loss of Bcl-2 was evident. The observed expression patterns of Bcl-2, Bax and Bcl-x protein in apoptotic lurcher and weaver neurons support the hypothesis that the execution of neuronal apoptosis involves increased expression of Bax, which could represent a general mechanism in diverse neurodegenerative processes.
我们研究了凋亡调节蛋白Bcl-2、Bax和Bcl-x在突变型蹒跚和织工小鼠小脑组织中的表达情况。在出生后小脑发育过程中，蹒跚小鼠的浦肯野细胞和织工小鼠的生发细胞（颗粒神经元前体细胞）均通过凋亡途径死亡。在实际细胞死亡过程发生之前，未检测到表达模式的显著变化。相反，凋亡的蹒跚小鼠浦肯野细胞中Bax和Bcl-x表达增加，而存活细胞的表达模式与健康同窝小鼠相似。在凋亡的织工小鼠生发细胞中也发现Bax表达增加，而未检测到Bcl-x表达的变化。Bcl-2在小脑神经元中低水平表达，且未发现Bcl-2缺失。在凋亡的蹒跚和织工神经元中观察到的Bcl-2、Bax和Bcl-x蛋白表达模式支持了以下假说：神经元凋亡的发生涉及Bax表达增加，这可能是多种神经退行性过程中的一种普遍机制。
