Shaw Christopher A, Pelech Steven, Ly Philip T T
Department of Neuroscience, The University of British Columbia, Vancouver, British Columbia, Canada.
Neurobiol Lipids. 2009 Jan 15;8(1):1-5.
The causes of many sporadic neurodegenerative diseases remain unknown making prevention difficult, if not impossible. One clue comes from the study of amyotrophic lateral sclerosis-parkinsonism dementia complex (ALS-PDC) of Guam which shares many similarities with amyotrophic lateral sclerosis, Parkinson's disease, and Alzheimer's disease seen in other parts of the world. This disorder may provide a unique opportunity to study the cause and progression of neurodegenerative diseases. Epidemiological and experimental findings indicate that dietary consumption of cycad seeds is an underlying cause of ALS-PDC. Our laboratory provided evidence that a family of compounds called steryl glycosides are the active ingredients that may be responsible for producing the neurodegenerative outcome in ALS-PDC. Here, we review some of our work on the chronic toxicity of steryl glycosides in neuronal cells maintained in cell culture and in an in vivo mouse model. The current studies indicate some mechanisms about how neuronal cells respond to this class of toxins.
许多散发性神经退行性疾病的病因仍然不明，这使得预防即便不是不可能，也变得极为困难。一条线索来自对关岛肌萎缩侧索硬化-帕金森病痴呆综合征（ALS-PDC）的研究，该综合征与世界其他地区所见的肌萎缩侧索硬化症、帕金森病和阿尔茨海默病有许多相似之处。这种疾病可能为研究神经退行性疾病的病因和进展提供了一个独特的机会。流行病学和实验结果表明，食用苏铁种子是ALS-PDC的一个潜在病因。我们的实验室提供了证据，表明一类名为甾醇糖苷的化合物是可能导致ALS-PDC神经退行性结果的活性成分。在此，我们回顾了我们在细胞培养中维持的神经元细胞和体内小鼠模型中关于甾醇糖苷慢性毒性的一些研究工作。目前的研究揭示了神经元细胞对这类毒素作出反应的一些机制。
