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Int. J. Mol. Sci. 2017, 18(7), 1411; doi:10.3390/ijms18071411

The Nucleolus: In Genome Maintenance and Repair

1
Institute of Molecular Biology and Biotechnology, Foundation for Research and Technology-Hellas, Nikolaou Plastira 100, 70013 Heraklion, Crete, Greece
2
Department of Biology, University of Crete, Vassilika Vouton, 71409 Heraklion, Crete, Greece
*
Author to whom correspondence should be addressed.
Received: 30 May 2017 / Revised: 22 June 2017 / Accepted: 27 June 2017 / Published: 1 July 2017
(This article belongs to the Special Issue Mechanisms Leading to Genomic Instability)
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Abstract

The nucleolus is the subnuclear membrane-less organelle where rRNA is transcribed and processed and ribosomal assembly occurs. During the last 20 years, however, the nucleolus has emerged as a multifunctional organelle, regulating processes that go well beyond its traditional role. Moreover, the unique organization of rDNA in tandem arrays and its unusually high transcription rates make it prone to unscheduled DNA recombination events and frequent RNA:DNA hybrids leading to DNA double strand breaks (DSBs). If not properly repaired, rDNA damage may contribute to premature disease onset and aging. Deregulation of ribosomal synthesis at any level from transcription and processing to ribosomal subunit assembly elicits a stress response and is also associated with disease onset. Here, we discuss how genome integrity is maintained within nucleoli and how such structures are functionally linked to nuclear DNA damage response and repair giving an emphasis on the newly emerging roles of the nucleolus in mammalian physiology and disease. View Full-Text
Keywords: nucleolus; DNA repair; nucleus; ribosomal DNA nucleolus; DNA repair; nucleus; ribosomal DNA
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Tsekrekou, M.; Stratigi, K.; Chatzinikolaou, G. The Nucleolus: In Genome Maintenance and Repair. Int. J. Mol. Sci. 2017, 18, 1411.

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