CGG repeat RNA G-quadruplexes interact with FMRpolyG to cause neuronal dysfunction in fragile X-related tremor/ataxia syndrome-Reference-Cited by-同舟云学术

CGG repeat RNA G-quadruplexes interact with FMRpolyG to cause neuronal dysfunction in fragile X-related tremor/ataxia syndrome

Published:2021-01-15 Issue:3 Volume:7 Page:
ISSN:2375-2548
Container-title:Science Advances
language:en
Short-container-title:Sci. Adv.

Author:

Asamitsu Sefan¹^ORCID,Yabuki Yasushi¹²^ORCID,Ikenoshita Susumu¹³^ORCID,Kawakubo Kosuke¹²^ORCID,Kawasaki Moe¹²^ORCID,Usuki Shingo⁴,Nakayama Yuji⁵,Adachi Kaori⁶^ORCID,Kugoh Hiroyuki⁷⁸,Ishii Kazuhiro⁹^ORCID,Matsuura Tohru¹⁰,Nanba Eiji¹¹,Sugiyama Hiroshi¹²^ORCID,Fukunaga Kohji¹³,Shioda Norifumi¹²^ORCID

Affiliation:

1. Department of Genomic Neurology, Institute of Molecular Embryology and Genetics (IMEG), Kumamoto University, Kumamoto, Japan.

2. Graduate School of Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan.

3. Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.

4. Liaison Laboratory Research Promotion Center, Institute of Molecular Embryology and Genetics (IMEG), Kumamoto University, Kumamoto, Japan.

5. Division of Radioisotope Science, Research Initiative Center, Organization for Research Initiative and Promotion, Tottori University, Tottori, Japan.

6. Division of Genomic Science, Research Initiative Center, Organization for Research Initiative and Promotion, Tottori University, Tottori, Japan.

7. Department of Biomedical Science, Institute of Regenerative Medicine and Biofunction, Graduate School of Medical Science, Tottori University, Tottori, Japan.

8. Chromosome Engineering Research Center, Tottori University, Tottori, Japan.

9. Department of the Neurology, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.

10. Division of Neurology, Department of Medicine, Jichi Medical University, Shimono, Japan.

11. Office for Research Strategy, Organization for Research Initiative and Promotion, Tottori University, Tottori, Japan.

12. Department of Chemistry, Graduate School of Science, Kyoto University, Kyoto, Japan.

13. Department of Pharmacology, Graduate School of Pharmaceutical Sciences, Tohoku University, Sendai, Japan.

Abstract

CGG repeat–derived RNA G-quadruplexes accelerate liquid-to-solid transition of FMRpolyG.

Funder

JSPS KAKENHI

AMED

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

Reference61 articles.

1. Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X

2. Fragile X-associated tremor/ataxia syndrome — features, mechanisms and management

3. Penetrance of the Fragile X–Associated Tremor/Ataxia Syndrome in a Premutation Carrier Population

4. RNA-Binding Proteins hnRNP A2/B1 and CUGBP1 Suppress Fragile X CGG Premutation Repeat-Induced Neurodegeneration in a Drosophila Model of FXTAS

5. Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients

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