Gene expression and DNA repair in progeroid syndromes and human aging
Abstract
Human progeroid syndromes are caused by mutations in single genes accelerating some but not all features of normal aging. Most progeroid disorders are linked to defects in genome maintenance, and while it remains unknown if similar processes underlie normal and premature aging, they provide useful models for the study of aging. Altered transcription is speculated to play a causative role in aging, and is involved in the pathology of most if not all progeroid syndromes. Previous studies demonstrate that there is a similar pattern of gene expression changes in primary cells from old and Werner syndrome compared to young suggesting a presence of common cellular aging mechanisms in old and progeria. Here we review the role of transcription in progeroid syndromes and discuss the implications of similar transcription aberrations in normal and premature aging.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In HGPS/RD, histone modification and gene expression patterns change, and DNA damage increases with a loss of DNA repair efficiency.
“Functionally, histone modification and gene expression patterns change [8,34], and DNA damage increases with a loss of DNA repair efficiency [12,18].”
From Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A (2009) · cites this paper as [8]
The citation lists haven’t been fetched yet
References 79 · Cited by 71
- Authors
- Kyng, K. J., & Bohr, V. A.
- Affiliations
- National Institutes of Health (US)
- Corresponding author
- Bohr, Vilhelm A.
- Venue
- Ageing Research Reviews
- Publisher
- Elsevier BV
- Published
- 2005-11
- Volume
- 4
- Issue
- 4
- Pages
- 579-602
- ISSN
- 1568-1637 (ISSN-L)
- OpenAlex
- W2065932894
- Cited by
- 71
- References
- 79
- Citations by year
- 2026: 3 · 2025: 2 · 2024: 3 · 2023: 1 · 2022: 1 · 2020: 3 · 2018: 1 · 2017: 1 · 2016: 2 · 2015: 1 · 2014: 1 · 2013: 4 · 2012: 5
- FWCI
- 0.66
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Ageing Research Reviews (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Kræftens Bekæmpelse
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Aging › Genetics, Aging, and Longevity in Model Organisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
- Health Sciences › Medicine › Physiology › Telomeres, Telomerase, and Senescence
- Keywords
- progeroid syndromes, Werner syndrome, Hutchinson-Gilford progeria syndrome, premature aging, gene expression, DNA repair, genome maintenance, transcription, cellular aging
- MeSH terms
- Aging, Animals, Cockayne Syndrome, DNA, DNA Damage, DNA Repair, Humans, Mutation, Progeria, Transcription, Genetic, Werner Syndrome, Gene Expression, Oligonucleotide Array Sequence Analysis
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
