Bonfanti L, Strettoi E, Chierzi S, Cenni M C, Liu X H, Maffei L, Rabacchi S A
Istituto di Neurofisiologia del Consiglio Nazionale delle Ricerche, Pisa, Italy.
J Neurosci. 1996 Jul 1;16(13):4186-94. doi: 10.1523/JNEUROSCI.16-13-04186.1996.
Approximately half of the retinal ganglion cells (RGCs) present in the rodent retina at birth normally die during early development. Overexpression of the photo-oncogene bcl-2 recently has been shown to rescue some neuronal populations from natural cell death and from degeneration induced by axotomy of nerves within the peripheral nervous system. Here we study in vivo the role of the overexpression of bcl-2 in the natural cell death of RGCs and in the degenerative process induced in these cells by transection of the optic nerve. We find that in newborn bcl-2 transgenic mice, the number of RGCs undergoing natural cell death is considerably lower than in wild-type pups. Consistently, a vast majority (90%) of the ganglion cells found in the retina of neonatal transgenics are maintained in adulthood, whereas only 40% survive in wild-type mice. After transection of the optic nerve, the number of degenerating ganglion cells, determined by counting pyknotic nuclei or nuclei with fragmented DNA, is substantially reduced in transgenic mice. In wild-type animals, almost 50% of ganglion cells degenerate in the 24 hr after the lesion, whereas almost the entire ganglion cell population survives axotomy in transgenic mice. Therefore, overexpression of bcl-2 is effective in preventing degeneration of this neuronal population, raising the possibility that ganglion cells are dependent on the endogenous expression of bcl-2 for survival. The remarkable rescue capacity of bcl-2 overexpression in these neurons makes it an interesting model for studying natural cell death and responses to injury in the CNS.
出生时存在于啮齿动物视网膜中的视网膜神经节细胞(RGCs)约有一半在早期发育过程中正常死亡。最近有研究表明,光癌基因bcl-2的过表达可使一些神经元群体免于自然细胞死亡以及外周神经系统内神经轴突切断诱导的退化。在此,我们在体内研究bcl-2过表达在RGCs自然细胞死亡以及视神经横断诱导的这些细胞退化过程中的作用。我们发现,在新生的bcl-2转基因小鼠中,经历自然细胞死亡的RGCs数量明显低于野生型幼崽。同样,在新生转基因小鼠视网膜中发现的绝大多数(90%)神经节细胞在成年期得以保留,而在野生型小鼠中只有40%存活。视神经横断后,通过计数固缩核或DNA片段化的核来确定的退化神经节细胞数量在转基因小鼠中大幅减少。在野生型动物中,损伤后24小时内几乎50%的神经节细胞退化,而在转基因小鼠中几乎整个神经节细胞群体在轴突切断后存活。因此,bcl-2的过表达有效地防止了这个神经元群体的退化,这增加了神经节细胞依赖bcl-2的内源性表达来存活的可能性。bcl-2过表达在这些神经元中显著的挽救能力使其成为研究中枢神经系统自然细胞死亡和对损伤反应的一个有趣模型。