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运动神经元病的遗传结构。

Genetic architecture of motor neuron diseases.

机构信息

Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow 226025, UP, India.

Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow 226025, UP, India.

出版信息

J Neurol Sci. 2022 Mar 15;434:120099. doi: 10.1016/j.jns.2021.120099. Epub 2021 Dec 22.

Abstract

Motor neuron diseases (MNDs) are rare and frequently fatal neurological disorders in which motor neurons within the brainstem and spinal cord regions slowly die. MNDs are primarily caused by genetic mutations, and > 100 different mutant genes in humans have been discovered thus far. Given the fact that many more MND-related genes have yet to be discovered, the growing body of genetic evidence has offered new insights into the diverse cellular and molecular mechanisms involved in the aetiology and pathogenesis of MNDs. This search may aid in the selection of potential candidate genes for future investigation and, eventually, may open the door to novel interventions to slow down disease progression. In this review paper, we have summarized detailed existing research findings of different MNDs, such as amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), spinal bulbar muscle atrophy (SBMA) and hereditary spastic paraplegia (HSP) in relation to their complex genetic architecture.

摘要

运动神经元病(MNDs)是一种罕见且常致命的神经系统疾病,其中脑干和脊髓区域的运动神经元会逐渐死亡。MNDs 主要由基因突变引起,迄今为止,人类已经发现了 >100 种不同的突变基因。鉴于还有更多与 MND 相关的基因尚未被发现,越来越多的遗传证据为 MND 的病因和发病机制涉及的多种细胞和分子机制提供了新的见解。这一探索可能有助于选择未来研究的潜在候选基因,并最终为减缓疾病进展开辟新的干预途径。在这篇综述论文中,我们总结了不同 MND(如肌萎缩侧索硬化症(ALS)、脊髓性肌萎缩症(SMA)、脊髓延髓肌肉萎缩症(SBMA)和遗传性痉挛性截瘫(HSP))与其复杂遗传结构相关的详细现有研究结果。

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