Apoptosis as the mechanism of neurodegeneration in Batten's disease.
Apoptosis as the mechanism of neurodegeneration in Batten's disease.
复制标题
细胞凋亡作为巴顿病神经变性的机制。
DOI：
10.1046/j.1471-4159.1996.67020677.x
复制
发表时间：
1996
影响因子：
4.7
通讯作者：
Boustany,RM
中科院分区：
文献类型：
--
作者：
Lane,SC;Jolly,RD;Schmechel,DE;Alroy,J;Boustany,RM
Batten's disease is a genetic neurodegenerative disease of childhood. Its hallmarks are retinitis pigmentosa and neuronal degeneration. As some types of photoreceptor death in mice are mediated by apoptosis, we investigated whether apoptosis is responsible for retinal and neuronal degeneration in the late infantile and juvenile forms of Batten's disease. Using the terminal dUDP nick end‐labeling (TUNEL) staining method, we detected apoptotic neuronal cells in brain from patients and a canine model and in brain and retina from an ovine model for Batten's disease. We confirmed apoptosis by flow cytometry, electron microscopy, and DNA laddering. This is the first inherited neurodegenerative disease involving brain and retina in which apoptosis has been established as the mechanism of neuronal and photoreceptor cell death in both humans and animal models.
