铁在肌萎缩侧索硬化症中的作用。
The Role of Iron in Amyotrophic Lateral Sclerosis.
机构信息
Department of Neurology and Centre for Clinical Neuroscience, Daping Hospital, Third Military Medical University, Chongqing, China.
Department of Neurology, Chengdu Military General Hospital, Chengdu, China.
出版信息
Adv Exp Med Biol. 2019;1173:145-152. doi: 10.1007/978-981-13-9589-5_8.
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.
肌萎缩侧索硬化症（ALS）是一种进行性神经退行性疾病，其特征是运动皮层、脑干和脊髓中的运动神经元退化。这种毁灭性疾病的病因和发病机制在很大程度上仍然未知。越来越多的证据表明，铁积累与 ALS 的发病和进展有关。在这篇综述中，我们讨论了大脑中铁稳态的调节、ALS 中铁稳态的失调及其在疾病机制中的可能作用。最后，我们总结了最近关于 ALS 的铁螯合剂治疗的进展和问题，旨在提出一种新的治疗策略来改善疾病的进展。
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