Interaction of the Transcription Factors USF1, USF2, and α-Pal/Nrf-1 with the FMR1 Promoter
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Hypermethylation of the FMR1 promoter reduces its transcriptional activity, resulting in the mental retardation and macroorchidism characteristic of Fragile X syndrome. How exactly methylation causes transcriptional silencing is not known but is relevant if current attempts to reactivate the gene are to be successful. Understanding the effect of methylation requires a better understanding of the factors responsible for FMR1 gene expression. To this end we have identified five evolutionarily conserved transcription factor binding sites in this promoter and shown that four of them are important for transcriptional activity in neuronally derived cells. We have also shown that USF1, USF2, and alpha-Pal/Nrf-1 are the major transcription factors that bind the promoter in brain and testis extracts and suggest that elevated levels of these factors account in part for elevated FMR1 expression in these organs. We also show that methylation abolishes alpha-Pal/Nrf-1 binding to the promoter and affects binding of USF1 and USF2 to a lesser degree. Methylation may therefore inhibit FMR1 transcription not only by recruiting histone deacetylases but also by blocking transcription factor binding. This suggests that for efficient reactivation of the FMR1 promoter, significant demethylation must occur and that current approaches to gene reactivation using histone deacetylase inhibitors alone may therefore have limited effect.
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These epigenetic changes promote a heterochromatic configuration that excludes specific transcription factors and turns gene expression off.
“These epigenetic changes promote a heterochromatic configuration that excludes the binding of specific transcription factors (19), thus turning gene expression off (20).”
From Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics (2012) · cites this paper as [19]
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Mogren, M. A., Teniou, A., Rhouati, A., Thakur, A., Alanazi, S., Anasori, B., & Zourob, M. M. (2025) · International Journal of Biological Macromolecules · doi:10.1016/j.ijbiomac.2025.145968 · Cited by 1 · see citing works
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Differentiation of neural stem cells in fragile X syndrome
Tervonen, T. A. (2008) · Työväentutkimus Vuosikirja
Nucleic Acid Structures and the Transcription Defects in Fragile X Syndrome and Friedreich’s Ataxia
Usdin, K. (2007) · Springer eBooks · doi:10.1007/0-387-29148-2_9
Self-citationHistory Tends to Repeat: FMR-1 Silencing in Fragile X Syndrome
Haas, J. G. (2007) · Eukaryon
Kapanadze, N. (2006) · PubMed
Gray, S. J. (2006) · Europe PMC (PubMed Central)
Tabolacci, E., & Chiurazzi, P. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-804461-2.00017-2
Markkanen, M. A. (2018) · Työväentutkimus Vuosikirja
Molecular Correlates of Fragile X Syndrome and FXTAS
Tassone, F., & Hagerman, P. J. (2007) · Springer eBooks · doi:10.1007/3-540-33336-3_2
- Authors
- Kumari, D., & Usdin, K.
- Affiliations
- National Institutes of Health (US)
- Corresponding author
- Usdin, Karen
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 2001-02
- Volume
- 276
- Issue
- 6
- Pages
- 4357-4364
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W2069894990
- Cited by
- 92
- References
- 28
- Citations by year
- 2025: 1 · 2023: 4 · 2021: 5 · 2020: 3 · 2019: 5 · 2018: 3 · 2017: 1 · 2016: 4 · 2015: 1 · 2014: 6 · 2013: 5 · 2012: 3
- FWCI
- 4.00
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Biological Chemistry (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health
- National Heart, Lung, and Blood Institute
- National Institute of Diabetes and Digestive and Kidney Diseases
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Epigenetics and DNA Methylation
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Genomics and Chromatin Dynamics
- Keywords
- USF1, USF2, DNA methylation, transcriptional silencing, fragile X syndrome, transcription factor binding sites, histone deacetylase, gene reactivation, histone deacetylase inhibitors, brain, testis
- MeSH terms
- Animals, Base Sequence, DNA, DNA-Binding Proteins, Fragile X Syndrome, Humans, Molecular Sequence Data, Phylogeny, Promoter Regions, Genetic, Protein Binding, Sequence Homology, Nucleic Acid, Transcription Factors, Trans-Activators, DNA Footprinting, DNA Methylation, Nuclear Respiratory Factors, Nuclear Respiratory Factor 1, Mice, Sp3 Transcription Factor, Upstream Stimulatory Factors
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