Ke Ya, Qian Zhong Ming
Department of Physiology, Faculty of Medicine, The Chinese University of Hong Kong, NT, Hong Kong.
Prog Neurobiol. 2007 Oct;83(3):149-73. doi: 10.1016/j.pneurobio.2007.07.009. Epub 2007 Aug 7.
New findings obtained during the past years, especially the discovery of mutations in the genes associated with brain iron metabolism, have provided key insights into the homeostatic mechanisms of brain iron metabolism and the pathological mechanisms responsible for neurodegenerative diseases. The accumulated evidence demonstrates that misregulation in brain iron metabolism is one of the initial causes for neuronal death in some neurodegenerative disorders. The errors in brain iron metabolism found in these disorders have a multifactorial pathogenesis, including genetic and nongenetic factors. The disturbances of iron metabolism might occur at multiple levels, including iron uptake and release, storage, intracellular metabolism and regulation. It is the increased brain iron that triggers a cascade of deleterious events, leading to neuronal death in these diseases. In the article, the recent advances in studies on neurochemistry and neuropathophysiology of brain iron metabolism were reviewed.
过去几年获得的新发现，尤其是与脑铁代谢相关基因中突变的发现，为脑铁代谢的稳态机制以及神经退行性疾病的病理机制提供了关键见解。积累的证据表明，脑铁代谢失调是某些神经退行性疾病中神经元死亡的初始原因之一。这些疾病中发现的脑铁代谢错误具有多因素发病机制，包括遗传和非遗传因素。铁代谢紊乱可能发生在多个层面，包括铁的摄取和释放、储存、细胞内代谢和调节。正是脑铁增加引发了一系列有害事件，导致这些疾病中的神经元死亡。本文综述了脑铁代谢的神经化学和神经病理生理学研究的最新进展。
