Control of CNS functions by RNA-binding proteins in neurological diseases.
Control of CNS functions by RNA-binding proteins in neurological diseases.
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DOI：
10.1007/s40495-018-0140-7
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发表时间：
2018-08
影响因子：
--
通讯作者：
Mao Y
中科院分区：
文献类型：
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作者：
Zhou Y;Dong F;Mao Y
本文综述了近年来关于RNA结合蛋白（RBP）在各种神经发育和神经退行性疾病（包括自闭症谱系障碍、精神分裂症、阿尔茨海默病、肌萎缩侧索硬化、额颞叶痴呆和脊髓小脑共济失调）中控制神经功能和发病机制的研究进展。RBP是基因表达的关键参与者，调节转录后修饰的每一步。最近的全基因组方法显示，许多蛋白质与RNA相关，但不包含任何已知的RNA结合基序。此外，许多神经发育和神经退行性疾病的致病基因和风险基因都是RBP。高通量测序方法的发展已经绘制出转录本上RBP的指纹图谱，并为发现神经系统疾病的新机制提供了前所未有的潜力。深入了解RBP如何调节神经发育对于设计许多神经发育和神经退行性疾病的有效疗法非常重要。RBP具有多种调节RNA加工的机制，从而控制神经发生。了解疾病相关RBP在神经发生中的作用对于开发神经系统疾病的新疗法至关重要。
This review summarizes recent studies on the molecular mechanisms of RNA binding proteins (RBPs) that control neurological functions and pathogenesis in various neurodevelopmental and neurodegenerative diseases, including autism spectrum disorders, schizophrenia, Alzheimer’s disease, amyotrophic lateral sclerosis, frontotemporal dementia, and spinocerebellar ataxia. RBPs are critical players in gene expression that regulate every step of posttranscriptional modifications. Recent genome-wide approaches revealed that many proteins associate with RNA, but do not contain any known RNA binding motifs. Additionally, many causal and risk genes of neurodevelopmental and neurodegenerative diseases are RBPs. Development of high-throughput sequencing methods has mapped out the fingerprints of RBPs on transcripts and provides unprecedented potential to discover new mechanisms of neurological diseases. Insights into how RBPs modulate neural development are important for designing effective therapies for numerous neurodevelopmental and neurodegenerative diseases. RBPs have diverse mechanisms for modulating RNA processing and, thereby, controlling neurogenesis. Understanding the role of disease-associated RBPs in neurogenesis is vital for developing novel treatments for neurological diseases.