The Role of Iron in Amyotrophic Lateral Sclerosis
The Role of Iron in Amyotrophic Lateral Sclerosis
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DOI：
10.1007/978-981-13-9589-5_8
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发表时间：
2019-01-01
期刊：
影响因子：
--
通讯作者：
Guo, Yansu
中科院分区：
文献类型：
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作者：
Bu, Xian-Le;Xiang, Yang;Guo, Yansu
肌萎缩侧索硬化症（ALS）是一种进行性神经退行性疾病，其特征是运动皮质、脑干和脊髓中的运动神经元变性。这种毁灭性疾病的病因和发病机制仍然在很大程度上未知。越来越多的证据表明，铁积累参与ALS的发病和进展。本文就脑内铁稳态的调节、ALS中铁稳态的失调及其在发病机制中的作用作一综述。最后，我们总结了铁螯合剂治疗ALS的最新进展和存在的问题，旨在提出一种新的治疗策略，以改善疾病的进展。
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the degeneration of motor neurons in the motor cortex, brainstem, and spinal cord. The etiology and pathogenesis of this devastating disease remain largely unknown. Increasing evidence suggests that iron accumulation is involved in the onset and progression of ALS. In this review, we discuss the regulation of iron homoeostasis in the brain, the misregulation of iron homeostasis in ALS, and its possible roles in the mechanism of the disease. Finally, we summarize the recent progress and problems with respect to iron chelator therapies on ALS, aiming to propose a new therapeutic strategy to ameliorate the progression of the disease.