ArticleNucleic acids research2004
Destabilization of tetraplex structures of the fragile X repeat sequence (CGG)n is mediated by homolog-conserved domains in three members of the hnRNP family.
Article in Nucleic acids research, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 67 citations in OpenAlex.
- Detangling knots: the intricate roles of G-quadruplexes in herpesvirus replication.Journal of virology · 2026Review
- Three- and four-stranded nucleic acid structures and their ligands.RSC chemical biology · 2025Review
- Trans-cis isomerization kinetics of cyanine dyes reports on the folding states of exogeneous RNA G-quadruplexes in live cells.Nucleic acids research · 2023Article
- Recent advances in applying G-quadruplex for SARS-CoV-2 targeting and diagnosis: A review.International journal of biological macromolecules · 2022Review
- Mechanisms of theInternational journal of molecular sciences · 2022Review
- (Dys)function Follows Form: Nucleic Acid Structure, Repeat Expansion, and Disease Pathology inInternational journal of molecular sciences · 2021Review
- The Cellular Functions and Molecular Mechanisms of G-Quadruplex Unwinding Helicases in Humans.Frontiers in molecular biosciences · 2021Review
- LANA and hnRNP A1 Regulate the Translation of LANA mRNA through G-Quadruplexes.Journal of virology · 2020Article
- Curcumin Regulates the r(CGG)Frontiers in neuroscience · 2020Article
- Bulged and Canonical G-Quadruplex Conformations Determine NDPK Binding Specificity.Molecules (Basel, Switzerland) · 2019Article
- Repeat-associated non-AUG (RAN) translation and other molecular mechanisms in Fragile X Tremor Ataxia Syndrome.Brain research · 2018Review
- Sorting mRNA Molecules for Cytoplasmic Transport and Localization.Frontiers in genetics · 2018Review
- Human DNA Helicase B as a Candidate for Unwinding Secondary CGG Repeat Structures at theFrontiers in molecular neuroscience · 2018Article
- RNA G-Quadruplexes in Biology: Principles and Molecular Mechanisms.Journal of molecular biology · 2017Review
- The cellular protein hnRNP A2/B1 enhances HIV-1 transcription by unfolding LTR promoter G-quadruplexes.Scientific reports · 2017Article
- RNA G-quadruplexes: emerging mechanisms in disease.Nucleic acids research · 2017Article
- Structural Characteristics of Simple RNA Repeats Associated with Disease and their Deleterious Protein Interactions.Frontiers in cellular neuroscience · 2017Review
- G-quadruplexes in pathogens: a common route to virulence control?PLoS pathogens · 2015Review
- Repeat-associated non-AUG translation and its impact in neurodegenerative disease.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2014Review
- DNA and RNA quadruplex-binding proteins.International journal of molecular sciences · 2014Review
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
Hairpin or tetrahelical structures formed by a d(CGG)n sequence in the FMR1 gene are thought to promote expansion of the repeat tract. Subsequent to this expansion FMR1 is silenced and fragile X syndrome ensues. The injurious effects of d(CGG)n secondary structures may potentially be countered by agents that act to decrease their stability. We showed previously that the hnRNP-related protein CBF-A destabilized G'2 bimolecular tetraplex structures of d(CGG)n. Analysis of mutant proteins revealed that the CBF-A-conserved domains RNP11 and ATP/GTP binding box were sufficient and necessary for G'2 d(CGG)n disruption while the RNP21 motif inhibited the destabilization activity. Here, we report that a C-terminal fragment of CBF-A whose only remaining conserved domain was the ATP/GTP binding motif, disrupted G'2 d(CGG)n more selectively than wild-type CBF-A. Further, two additional members of the hnRNP family, hnRNP A2 and mutant hnRNP A1 effectively destabilized G'2 d(CGG)n. Examination of mutant hnRNP A2 proteins revealed that, similar to CBF-A, their RNP11 element and ATP/GTP binding motif mediated G'2 d(CGG)n disruption, while the RNP21 element blocked their action. Similarly, the RNP11 and RNP21 domains of hnRNP A1 were, respectively, positive and negative mediators of G'2 d(CGG)n destabilization. Last, employing the same conserved motifs that mediated disruption of the DNA tetraplex G'2 d(CGG)n, hnRNP A2 destabilized r(CGG)n RNA tetraplex.
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