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Short Tandem Repeat-Enriched Architectural RNAs in Nuclear Bodies: Functions and Associated Diseases

Institute for Genetic Medicine, Hokkaido University, Sapporo 060-0815, Japan
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Non-Coding RNA 2020, 6(1), 6; https://doi.org/10.3390/ncrna6010006
Received: 10 January 2020 / Revised: 31 January 2020 / Accepted: 19 February 2020 / Published: 20 February 2020
(This article belongs to the Special Issue Non-Coding RNA and Intracellular Structures)
Nuclear bodies are membraneless, phase-separated compartments that concentrate specific proteins and RNAs in the nucleus. They are believed to serve as sites for the modification, sequestration, and storage of specific factors, and to act as organizational hubs of chromatin structure to control gene expression and cellular function. Architectural (arc) RNA, a class of long noncoding RNA (lncRNA), plays essential roles in the formation of nuclear bodies. Herein, we focus on specific arcRNAs containing short tandem repeat-enriched sequences and introduce their biological functions and recently elucidated underlying molecular mechanism. In various neurodegenerative diseases, abnormal nuclear and cytoplasmic bodies are built on disease-causing RNAs or toxic RNAs with aberrantly expanded short tandem repeat-enriched sequences. We discuss the possible analogous functions of natural arcRNAs and toxic RNAs with short tandem repeat-enriched sequences. Finally, we describe the technical utility of short tandem repeat-enriched arcRNAs as a model for exploring the structures and functions of nuclear bodies, as well as the pathogenic mechanisms of neurodegenerative diseases. View Full-Text
Keywords: architectural RNA; nuclear body; short tandem repeat-enriched RNA; repeat-expansion disease; toxic RNA architectural RNA; nuclear body; short tandem repeat-enriched RNA; repeat-expansion disease; toxic RNA
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Ninomiya, K.; Hirose, T. Short Tandem Repeat-Enriched Architectural RNAs in Nuclear Bodies: Functions and Associated Diseases. Non-Coding RNA 2020, 6, 6.

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