肌萎缩侧索硬化(ALS)的治疗干预措施

IF 16.4 1区 化学 Q1 CHEMISTRY, MULTIDISCIPLINARY Accounts of Chemical Research Pub Date : 2022-08-01 DOI:10.33425/2692-7918.1032
A. Chakraborty, Anil Diwan
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引用次数: 0

摘要

肌萎缩性侧索硬化症(ALS)是一种非脱髓鞘性神经退行性疾病,常见于40至60岁的成年人。这种疾病通常在男性中流行,但与不同性别无关。ALS是一种进行性疾病,在诊断后2-5年内最终以死亡告终。大多数ALS病例是散发的(90%),没有任何明确的病因。另外10-12%的病例是由20多种基因突变引起的。据报道,导致ALS的基因有超氧化物歧化酶1 (SOD1)、TAR DNA结合蛋白(TDP)、肉瘤融合(FUS)、9号染色体开放阅读框72 (c9orf72)和囊泡相关膜蛋白相关蛋白B (VAPB)。此外,ALS患者的脂质代谢异常,LDL/HDL比值较高。ALS的病因如下图所示(图1)。由于ALS的致病因素和症状具有多重性质,目前还没有针对ALS的特异性治疗方法。然而,本文将触及潜在的治疗方法,在实践中或可能出现在未来。目标是维持和改善运动神经元的功能,改善ALS患者的健康和生活质量。在此之前,我们必须依靠对症治疗和康复措施来支持患者的生活质量。
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Therapeutic interventions of Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic lateral sclerosis (ALS) is a non-demyelinating neurodegenerative disease mostly found in adults between 40 to 60 years old. This disease is usually prevalent in males, however it’s irrespective to the different genders. ALS is progressive and within 2-5 years of diagnosis ulimately ends with death. The majority of ALS cases is sporadic (90%) and is recorded without any defined aetiology. The other 10-12% of cases is recognized from mutations in more than 20 genes. The genes reported to cause ALS are Superoxide Dismutase 1 (SOD1), TAR DNA Binding Protein (TDP), Fused in Sarcoma, (FUS), Chromosome 9 Open Reading Frame 72 (c9orf72) and Vesicle-Associated Membrane-ProteinAssociated Protein B (VAPB). Furthermore, abnormal lipid metabolism with higher LDL/HDL ratio was reported in ALS patients. The aetiology of ALS is shown in the schematic diagram below (Figure 1) Due to the multi-nature of ALS causative factors and symptoms, there is no specific therapy for ALS today. However, this paper will touch on potential therapies that are in practice or may come up in the future. The goal is to maintain and improve the function of motor neuron, the well-being and quality of life for ALS patients. Until then, we have to rely on the symptomatic treatment and rehabilitative measures to support the patient’s quality of life.
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来源期刊
Accounts of Chemical Research
Accounts of Chemical Research 化学-化学综合
CiteScore
31.40
自引率
1.10%
发文量
312
审稿时长
2 months
期刊介绍: Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.
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