Gemin3, also known as DDX20 or DP103, is a DEAD-box RNA helicase which is involved in more than one cellular process. Though RNA unwinding has been determined in vitro, it is surprisingly not required for all of its activities in cellular metabolism. Gemin3 is an essential gene, present in Amoeba and Metazoa. The highly conserved N-terminus hosts the helicase core, formed of the helicase- and DEAD-domains, which, based on crystal structure determination, have key roles in RNA binding. The C-terminus of Gemin3 is highly divergent between species and serves as the interaction site for several accessory factors that could recruit Gemin3 to its target substrates and/or modulate its function. This review article focuses on the known roles of Gemin3, first as a core member of the survival motor neuron (SMN) complex, in small nuclear ribonucleoprotein biogenesis. Although mechanistic details are lacking, a critical function for Gemin3 in this pathway is supported by numerous in vitro and in vivo studies. Gene expression activities of Gemin3 are next underscored, mainly messenger ribonucleoprotein trafficking, gene silencing via microRNA processing, and transcriptional regulation. The involvement of Gemin3 in abnormal cell signal transduction pathways involving p53 and NF-κB is also highlighted. Finally, the clinical implications of Gemin3 deregulation are discussed including links to spinal muscular atrophy, poliomyelitis, amyotrophic lateral sclerosis, and cancer. Impressive progress made over the past two decades since the discovery of Gemin3 bodes well for further work that refines the mechanism(s) underpinning its multiple activities.
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April 2018
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A 3D rendering of a Ubiquitin protein molecule. In this issue of Biochemical Society Transactions, Ovaa and Vertegaal discuss the role of ubiquitination and SUMO proteins in conjugation and deconjugation machineries; for details, see pages 423–436.
Review Article|
March 09 2018
The multiple lives of DEAD-box RNA helicase DP103/DDX20/Gemin3
Frank Curmi;
Frank Curmi
1Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
2Centre for Molecular Medicine and Biobanking, Biomedical Sciences Building, University of Malta, Msida, Malta
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Ruben J. Cauchi
1Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida, Malta
2Centre for Molecular Medicine and Biobanking, Biomedical Sciences Building, University of Malta, Msida, Malta
Correspondence: Ruben J. Cauchi (ruben.cauchi@um.edu.mt)
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Publisher: Portland Press Ltd
Received:
January 16 2018
Revision Received:
February 10 2018
Accepted:
February 15 2018
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© 2018 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society
2018
Biochem Soc Trans (2018) 46 (2): 329–341.
Article history
Received:
January 16 2018
Revision Received:
February 10 2018
Accepted:
February 15 2018
Citation
Frank Curmi, Ruben J. Cauchi; The multiple lives of DEAD-box RNA helicase DP103/DDX20/Gemin3. Biochem Soc Trans 17 April 2018; 46 (2): 329–341. doi: https://doi.org/10.1042/BST20180016
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