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将基因发现转化为对儿童运动障碍的机制理解:来自遗传性肌张力障碍及相关疾病的经验教训。

Translating Genetic Discovery into a Mechanistic Understanding of Pediatric Movement Disorders: Lessons from Genetic Dystonias and Related Disorders.

作者信息

Lin Wei-Sheng

机构信息

Department of Pediatrics Taipei Veterans General Hospital Taipei 11217 Taiwan.

School of Medicine National Yang Ming Chiao Tung University Taipei 112304 Taiwan.

出版信息

Adv Genet (Hoboken). 2022 Nov 9;4(2):2200018. doi: 10.1002/ggn2.202200018. eCollection 2023 Jun.

Abstract

The era of next-generation sequencing has increased the pace of gene discovery in the field of pediatric movement disorders. Following the identification of novel disease-causing genes, several studies have aimed to link the molecular and clinical aspects of these disorders. This perspective presents the developing stories of several childhood-onset movement disorders, including paroxysmal kinesigenic dyskinesia, myoclonus-dystonia syndrome, and other monogenic dystonias. These stories illustrate how gene discovery helps focus the research efforts of scientists trying to understand the mechanisms of disease. The genetic diagnosis of these clinical syndromes also helps clarify the associated phenotypic spectra and aids the search for additional disease-causing genes. Collectively, the findings of previous studies have led to increased recognition of the role of the cerebellum in the physiology and pathophysiology of motor control-a common theme in many pediatric movement disorders. To fully exploit the genetic information garnered in the clinical and research arenas, it is crucial that corresponding multi-omics analyses and functional studies also be performed at scale. Hopefully, these integrated efforts will provide us with a more comprehensive understanding of the genetic and neurobiological bases of movement disorders in childhood.

摘要

下一代测序时代加快了儿童运动障碍领域的基因发现步伐。在鉴定出新型致病基因后,多项研究旨在将这些疾病的分子和临床方面联系起来。本文观点介绍了几种儿童期起病的运动障碍的发展历程,包括发作性运动诱发性运动障碍、肌阵挛性肌张力障碍综合征和其他单基因肌张力障碍。这些历程说明了基因发现如何有助于集中科学家们试图理解疾病机制的研究工作。这些临床综合征的基因诊断也有助于阐明相关的表型谱,并有助于寻找其他致病基因。总体而言,先前研究的结果使人们越来越认识到小脑在运动控制的生理和病理生理中的作用——这是许多儿童运动障碍中的一个共同主题。为了充分利用在临床和研究领域获得的基因信息,至关重要的是还应大规模开展相应的多组学分析和功能研究。希望这些综合努力将使我们对儿童运动障碍的遗传和神经生物学基础有更全面的了解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6805/10242408/519d30de12e9/GGN2-4-2200018-g001.jpg

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