Sipe Jack C, Lee Pauline, Beutler Ernest
Department of Molecular and Experimental Medicine, Scripps Research Institute (MEM-215), La Jolla, CA 92037, USA.
Dev Neurosci. 2002;24(2-3):188-96. doi: 10.1159/000065701.
Iron, an essential element for central nervous system (CNS) function, has frequently been found to accumulate in brain regions that undergo degeneration in neurological diseases such as Alzheimer disease, Parkinson disease, Friedreich ataxia and other disorders. However, the precise role of iron in the cause of many neurodegenerative diseases is unclear. To assist in understanding the potential importance of iron in CNS disease, this review summarizes the present knowledge in the areas of CNS iron metabolism, homeostasis and disregulation of iron balance caused by mutations in genes encoding proteins involved in iron transport, storage and metabolism. This review encompasses neurodegenerative disorders associated with both iron overload and deficiency to highlight areas where iron misregulation is likely to be important in the pathophysiology of several human brain diseases.
铁是中枢神经系统（CNS）功能所必需的元素，人们经常发现它在神经疾病（如阿尔茨海默病、帕金森病、弗里德赖希共济失调和其他疾病）中发生退化的脑区中积累。然而，铁在许多神经退行性疾病病因中的具体作用尚不清楚。为了有助于理解铁在中枢神经系统疾病中的潜在重要性，本综述总结了中枢神经系统铁代谢、铁稳态以及由参与铁运输、储存和代谢的蛋白质编码基因突变导致的铁平衡失调等领域的现有知识。本综述涵盖了与铁过载和铁缺乏相关的神经退行性疾病，以突出铁调节异常在几种人类脑部疾病病理生理学中可能重要的领域。
