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DOI | 10.1126/science.aar7366 |
RNA buffers the phase separation behavior of prion-like RNA binding proteins | |
Maharana, Shovamayee1; Wang, Jie1; Papadopoulos, Dimitrios K.1,2; Richter, Doris1; Pozniakovsky, Andrey1; Poser, Ina1; Bickle, Marc1; Rizk, Sandra1,3; Guillen-Boixet, Jordina1; Franzmann, Titus M.1; Jahnel, Marcus1,4; Marrone, Lara5; Chang, Young-Tae6,7; Sterneckert, Jared5; Tomancak, Pavel1; Hyman, Anthony A.1; Alberti, Simon1 | |
2018-05-25 | |
发表期刊 | SCIENCE |
ISSN | 0036-8075 |
EISSN | 1095-9203 |
出版年 | 2018 |
卷号 | 360期号:6391页码:918-921 |
文章类型 | Article |
语种 | 英语 |
国家 | Germany; Scotland; South Korea |
英文摘要 | Prion-like RNA binding proteins (RBPs) such as TDP43 and FUS are largely soluble in the nucleus but form solid pathological aggregates when mislocalized to the cytoplasm. What keeps these proteins soluble in the nucleus and promotes aggregation in the cytoplasm is still unknown. We report here that RNAcritically regulates the phase behavior of prion-like RBPs. Low RNA/protein ratios promote phase separation into liquid droplets, whereas high ratios prevent droplet formation in vitro. Reduction of nuclear RNA levels or genetic ablation of RNA binding causes excessive phase separation and the formation of cytotoxic solid-like assemblies in cells. We propose that the nucleus is a buffered system in which high RNA concentrations keep RBPs soluble. Changes in RNA levels or RNA binding abilities of RBPs cause aberrant phase transitions. |
领域 | 地球科学 ; 气候变化 ; 资源环境 |
收录类别 | SCI-E |
WOS记录号 | WOS:000433039800051 |
WOS关键词 | AMYOTROPHIC-LATERAL-SCLEROSIS ; FUS MUTATIONS ; TOXIC GAIN ; TDP-43 ; ALS ; TRANSITION ; DEGENERATION ; MECHANISM ; DOMAINS ; DISEASE |
WOS类目 | Multidisciplinary Sciences |
WOS研究方向 | Science & Technology - Other Topics |
引用统计 | |
文献类型 | 期刊论文 |
条目标识符 | http://119.78.100.173/C666/handle/2XK7JSWQ/198737 |
专题 | 地球科学 资源环境科学 气候变化 |
作者单位 | 1.Max Planck Inst Mol Cell Biol & Genet, Pfotenhauerstr 108, D-01307 Dresden, Germany; 2.Univ Edinburgh, MRC Human Genet Unit, Inst Genet & Mol Med, Crewe Rd, Edinburgh EH4 2XU, Midlothian, Scotland; 3.Tech Univ Dresden, Cube Ctr Mol Bioengn B, Arnoldstr 18, D-01307 Dresden, Germany; 4.Tech Univ Dresden, Ctr Biotechnol, Tatzberg 47-49, D-01307 Dresden, Germany; 5.Tech Univ Dresden, CMCB, DFG Ctr Regenerat Therapies Dresden, D-01307 Dresden, Germany; 6.Inst for Basic Sci Korea, Ctr Self Assembly & Complex, Pohang 37673, South Korea; 7.Pohang Univ Sci & Technol POSTECH, Dept Chem, Pohang 37673, South Korea |
推荐引用方式 GB/T 7714 | Maharana, Shovamayee,Wang, Jie,Papadopoulos, Dimitrios K.,et al. RNA buffers the phase separation behavior of prion-like RNA binding proteins[J]. SCIENCE,2018,360(6391):918-921. |
APA | Maharana, Shovamayee.,Wang, Jie.,Papadopoulos, Dimitrios K..,Richter, Doris.,Pozniakovsky, Andrey.,...&Alberti, Simon.(2018).RNA buffers the phase separation behavior of prion-like RNA binding proteins.SCIENCE,360(6391),918-921. |
MLA | Maharana, Shovamayee,et al."RNA buffers the phase separation behavior of prion-like RNA binding proteins".SCIENCE 360.6391(2018):918-921. |
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