NEURONAL IRON IN HUNGTINGTON'S DISEASE

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 由亨廷顿蛋白基因内CAG扩增引起的神经退行性疾病。在美国，它影响着大约30000人。尽管自1993年发现该基因以来，我们对HD的认识有了很大的进步，但神经保护性治疗尚不存在。铁代谢异常与包括HD和帕金森病在内的神经退行性疾病的发病机制有关。理论主要集中在过量的铁促进氧化应激和细胞新生。然而，对这些过程的细胞和亚细胞位置以及为什么HD患者脑铁含量增加缺乏明确的了解。我们的初步数据表明，铁代谢的改变发生在HD的早期，铁代谢的特定调节具有神经保护作用。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. neurodegenerative disease that is caused by a CAG expansion within the huntingtin gene. It affects about 30000 people in the USA. Despite significant advances in our understanding of HD since identification of the gene in 1993, neuroprotective therapies are not available. Aberrant iron metabolism is implicated in the pathogenesis of neurodegenerative diseases including HD and Parkinson's disease. Theories have centered on excess iron promoting oxidative stress and cell negeneration. However, a clear understanding of the cell and subcellular locations of these processes as well as why brain iron is increased in HD is lacking. Our preliminary data suggests that alterations in iron metabolism occur early in HD and that specific modulation of iron metabolism is neuroprotective.