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2017 ; 45
(4
): 1584-1595
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RNA G-quadruplexes: emerging mechanisms in disease
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Cammas A
; Millevoi S
Nucleic Acids Res
2017[Feb]; 45
(4
): 1584-1595
PMID28013268
show ga
RNA G-quadruplexes (G4s) are formed by G-rich RNA sequences in protein-coding
(mRNA) and non-coding (ncRNA) transcripts that fold into a four-stranded
conformation. Experimental studies and bioinformatic predictions support the view
that these structures are involved in different cellular functions associated to
both DNA processes (telomere elongation, recombination and transcription) and RNA
post-transcriptional mechanisms (including pre-mRNA processing, mRNA turnover,
targeting and translation). An increasing number of different diseases have been
associated with the inappropriate regulation of RNA G4s exemplifying the
potential importance of these structures on human health. Here, we review the
different molecular mechanisms underlying the link between RNA G4s and human
diseases by proposing several overlapping models of deregulation emerging from
recent research, including (i) sequestration of RNA-binding proteins, (ii)
aberrant expression or localization of RNA G4-binding proteins, (iii) repeat
associated non-AUG (RAN) translation, (iv) mRNA translational blockade and (v)
disabling of protein-RNA G4 complexes. This review also provides a comprehensive
survey of the functional RNA G4 and their mechanisms of action. Finally, we
highlight future directions for research aimed at improving our understanding on
RNA G4-mediated regulatory mechanisms linked to diseases.
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