Posttranscriptional RNA stabilization of telomeric RNAs FRG2, DBE-T, D4Z4 at human 4q35 in response to genotoxic stress and D4Z4 macrosatellite repeat length

Our study reveals unforeseen biochemical features of RNAs from clustered transcription units within the 4q35 subtelomere. Specifically, the FRG2, DBE-T and D4Z4-derived transcripts are chromatin-associated and are stabilized posttranscriptionally after induction by genotoxic stress. Remarkably, the extent of this response is modulated by the copy number of the D4Z4 repeats, raising new hypotheses about their regulation and function in human biology and disease.

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Publication Details

Journal
Clinical Epigenetics
Published
2025-05-04
DOI
https://doi.org/10.1186/s13148-025-01881-5
Citations
3
Primary Topic
RNA regulation and disease
Type
article
Field-Weighted Citation Impact
2.42

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article

Posttranscriptional RNA stabilization of telomeric RNAs FRG2, DBE-T, D4Z4 at human 4q35 in response to genotoxic stress and D4Z4 macrosatellite repeat length

Paul D. Kaufman, Valentina Salsi, Monica Salani, Rossella Ginevra Tupler et al.
3 citations
Clinical Epigenetics
RNA regulation and disease
2.42
article

Posttranscriptional RNA stabilization of telomeric RNAs FRG2, DBE-T, D4Z4 at human 4q35 in response to genotoxic stress and D4Z4 macrosatellite repeat length

Paul D. Kaufman, Valentina Salsi, Monica Salani, Rossella Ginevra Tupler, Francesca Losi
article en
3 citations

Abstract

Our study reveals unforeseen biochemical features of RNAs from clustered transcription units within the 4q35 subtelomere. Specifically, the FRG2, DBE-T and D4Z4-derived transcripts are chromatin-associated and are stabilized posttranscriptionally after induction by genotoxic stress. Remarkably, the extent of this response is modulated by the copy number of the D4Z4 repeats, raising new hypotheses about their regulation and function in human biology and disease.

Clinical EpigeneticsVol. 17(1)
University of Modena and Reggio Emilia (IT), University of Massachusetts Chan Medical School (US), Massachusetts General Hospital (US), Center for Systems Biology (US)
FSHD Global Research Foundation, Ministero dell’Istruzione, dell’Università e della Ricerca, National Institutes of Health
Openalex Percentile: Top 12%
RNA regulation and disease
2.42
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