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神经罕见病的药物与机制洞察:现状与未来期望

Insights into the pharmaceuticals and mechanisms of neurological orphan diseases: Current Status and future expectations.

作者信息

Ramalho Teodorico C, de Castro Alexandre A, Tavares Tássia S, Silva Maria C, Silva Daniela R, Cesar Pedro H, Santos Lucas A, da Cunha Elaine F F, Nepovimova Eugenie, Kuca Kamil

机构信息

Department of Chemistry, Federal University of Lavras, 37200-000, Lavras, Brazil; Center for Basic and Applied Research, Faculty of Informatics and Management, University of Hradec Kralove, Hradec Kralove, Czech Republic.

Department of Chemistry, Federal University of Lavras, 37200-000, Lavras, Brazil.

出版信息

Prog Neurobiol. 2018 Oct;169:135-157. doi: 10.1016/j.pneurobio.2018.06.011. Epub 2018 Jul 4.

Abstract

Several rare or orphan diseases have been characterized that singly affect low numbers of people, but cumulatively reach ∼6%-10% of the population in Europe and in the United States. Human genetics has shown to be broadly effective when evaluating subjacent genetic defects such as orphan genetic diseases, but on the other hand, a modest progress has been achieved toward comprehending the molecular pathologies and designing new therapies. Chemical genetics, placed at the interface of chemistry and genetics, could be employed to understand the molecular mechanisms of subjacent illnesses and for the discovery of new remediation processes. This review debates current progress in chemical genetics, and how a variety of compounds and reaction mechanisms can be used to study and ultimately treat rare genetic diseases. We focus here on a study involving Amyotrophic lateral sclerosis (ALS), Duchenne Muscular Dystrophy (DMD), Spinal muscular atrophy (SMA) and Familial Amyloid Polyneuropathy (FAP), approaching different treatment methods and the reaction mechanisms of several compounds, trying to elucidate new routes capable of assisting in the treatment profile.

摘要

已经确定了几种罕见病或孤儿病,这些疾病单独影响的人数较少,但在欧洲和美国累计约占总人口的6%-10%。人类遗传学在评估诸如孤儿遗传病等潜在遗传缺陷时已被证明具有广泛的有效性,但另一方面,在理解分子病理学和设计新疗法方面进展甚微。化学遗传学处于化学和遗传学的交叉领域,可用于了解潜在疾病的分子机制并发现新的治疗方法。本综述讨论了化学遗传学的当前进展,以及如何使用各种化合物和反应机制来研究并最终治疗罕见遗传病。我们在此重点关注一项涉及肌萎缩侧索硬化症(ALS)、杜兴氏肌肉营养不良症(DMD)、脊髓性肌萎缩症(SMA)和家族性淀粉样多神经病(FAP)的研究,探讨不同的治疗方法和几种化合物的反应机制,试图阐明能够辅助治疗的新途径。

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