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首页> 外文期刊>Folia neuropathologica >Amyotrophic lateral sclerosis - looking for pathogenesis and effective therapy.
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Amyotrophic lateral sclerosis - looking for pathogenesis and effective therapy.

机译：肌萎缩性侧索硬化症-寻找发病机理和有效疗法。

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摘要

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by loss of motor neurons in the spinal cord, brain stem and motor cortex which dramatically reduces life expectancy. ALS occurs either in familial or, more frequently, in sporadic forms. It finally results in death due to respiratory failure that occurs typically 2-5 years after the disease onset. Although the aetiology of ALS remains largely unclear, its heterogeneity suggests that a combination of various factors, including endogenous and/or environmental ones, may be implicated in progressive motor neuron stress that results in the activation of different cell death pathways. Interactions between genetic, environmental, and age-dependent risk factors have been hypothesized to trigger disease onset. Despite extensive neurobiological, molecular and genetic research, at the beginning of the 21st century ALS still remains one of the most devastating neurodegenerative diseases because of the lack of effective therapeutic strategies. It is a challenge for the clinical and scientific community. Better understanding of the aetiology of amyotrophic lateral sclerosis is necessary to develop effective treatment of this progressive neurodegenerative disease. This review presents the current state of knowledge in ALS research.
机译：肌萎缩性侧索硬化症（ALS）是一种进行性神经退行性疾病，其特征在于脊髓，脑干和运动皮层中运动神经元的丢失，从而大大缩短了预期寿命。 ALS以家族形式或更普遍地以散发形式出现。最终由于呼吸衰竭导致死亡，这种死亡通常在疾病发作后2-5年发生。尽管ALS的病因学尚不清楚，但其异质性表明，各种因素的组合，包括内源性和/或环境因素，可能与进行性运动神经元应激有关，导致不同的细胞死亡途径被激活。假设遗传，环境和年龄相关危险因素之间的相互作用会触发疾病发作。尽管进行了广泛的神经生物学，分子和遗传学研究，但由于缺乏有效的治疗策略，在21世纪初ALS仍然是最严重的神经退行性疾病之一。对于临床和科学界来说，这是一个挑战。要发展对这种进行性神经退行性疾病的有效治疗，必须更好地了解肌萎缩性侧索硬化的病因。这篇综述介绍了ALS研究的当前知识状态。

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