脑铁代谢和神经变性性疾病

最近关于脑铁代谢研究的新成果，尤其是与脑铁转运、储存、调节相关的某些突变基因的发现，足以得出以下结论，即异常增高的脑铁至少是部份神经变性疾病的起始原因。研究显示，脑铁过量积聚主要是由于遗传性和非遗传性因素所引起的某些服铁代谢蛋白功能异常或表达失控。正是异常增高的脑铁触发一系列病理反应，最终导致神经为性性疾病病人服神经元死亡。本文简要叙述了目前对服铁分布、功能和脑铁代谢蛋白的认识，讨论了内铁转运机制以及服铁和神经变性性疾病之间的关系研究的新进展。

Brain Iron Metabolism and Neurodegenerative Diseases

New findings from recent studies on brain iron metabolism, especially the discovery of mutations in the genes associated with iron transport, storage or regulation in the brain, are more than enough to conclude that the increased brain iron is an initial cause of neuronal death at least in some neurodegenerative diseases. The excess iron accumulation in the brain may lead by the disrupted expression or function of iron metabolism proteins induced by either genetic or nongenetic factors. It is the increased brain iron that triggers a cascade of deleterious events, leading to neuronal death in these disorders. In this article, the recent advances in the study on mechanisms of iron transport in the brain and the relationship between the increased brain iron and neurodegenerative disorders were discussed. Also, current knowledge on brain iron distribution and function was briefly reviewed.