Involvement of xeroderma pigmentosum group A (XPA) in progeria arising from defective maturation of prelamin A
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Cellular accumulation of DNA damage has been widely implicated in cellular senescence, aging, and premature aging. In Hutchinson‐Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD), premature aging is linked to accumulation of DNA double‐strand breaks (DSBs), which results in genome instability. However, how DSBs accumulate in cells despite the presence of intact DNA repair proteins remains unknown. Here we report that the recruitment of DSB repair factors Rad50 and Rad51 to the DSB sites, as marked by γ‐H2AX, was impaired in human HGPS and Zmpste24‐deficient cells. Consistently, the progeria‐associated DSBs appeared to be unrepairable although DSBs induced by camptothecin were efficiently removed in the progeroid cells. We also found that these progeroid cells exhibited nuclear foci of xeroderma pigmentosum group A (XPA), a unique nucleotide excision repair protein. Strikingly, these XPA foci colocalized with the DSB sites in the progeroid cells. This XPA‐DSB association was further confirmed and found to be mediated by DNA, using a modified chromatin immunoprecipitation assay and coimmunoprecipitation. RNA interference (RNAi) knockdown of XPA in HGPS cells partially restored DSB repair as evidenced by Western blot analysis, immunofluorescence and comet assays. We propose that the uncharacteristic localization of XPA to or near DSBs inhibits DSB repair, thereby contributing to the premature aging phenotypes observed in progeria arising from genetic defects in prelamin A maturation.—Liu, Y., Wang, Y., Rusinol, A. E., Sinensky, M. S., Liu, J., Shell, S. M., Zou, Y. Involvement of xeroderma pigmentosum group A (XPA) in progeria arising from defective maturation of prelamin A. FASEB J. 22, 603–611 (2008)
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XPA misaccumulates at DSB sites consisting of ds-ssDNA junctions even though XPA has no documented role in DSB repair, causing these breaks to become progressively devoid of DSB repair proteins.
“Interestingly, cellular nucleotide excision repair protein XPA misaccumulates at the DSB sites consisting of ds-ssDNA junctions even though XPA never has had a documented role in DSB repair, causing these breaks to become progressively devoid of DSB repair proteins 114.”
From Xeroderma Pigmentosa Group A (XPA), Nucleotide Excision Repair and Regulation by ATR in Response to Ultraviolet Irradiation (2017) · cites this paper as [114]
HGPS cells with progerin accumulation have a reduced replicative lifespan and a deficiency in repairing endogenous laminopathy-induced DNA double-strand breaks, which increase with age.
“HGPS cells with progerin accumulation exhibit a reduced replicative lifespan plus a deficiency in the repair of endogenous, laminopathy-induced DNA DSBs, which increase with age 113, 114.”
From Xeroderma Pigmentosa Group A (XPA), Nucleotide Excision Repair and Regulation by ATR in Response to Ultraviolet Irradiation (2017) · cites this paper as [114]
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- Authors
- Liu, Y., Wang, Y., Rusinol, A. E., Sinensky, M. S., Liu, J., Shell, S. M., & Zou, Y.
- Affiliations
- East Tennessee State University (US)
- Department of Biochemistry and Molecular Biology James H. Quillen College of Medicine East Tennessee State University Johnson City Tennessee USA
- Venue
- The FASEB Journal
- Publisher
- Wiley
- Published
- 2007-09-11
- Volume
- 22
- Issue
- 2
- Pages
- 603-611
- ISSN
- 0892-6638 (ISSN-L)
- 1530-6860
- PMC
- PMC3116236
- OpenAlex
- W2083296702
- Semantic Scholar
- 40bc83eb79d612dc82c6d2c0a0cfd8cac7daab11
- Cited by
- 115
- References
- 46
- Citations by year
- 2025: 5 · 2024: 3 · 2023: 6 · 2022: 7 · 2021: 4 · 2020: 2 · 2019: 3 · 2018: 8 · 2017: 8 · 2016: 8 · 2015: 9 · 2014: 8 · 2013: 9 · 2012: 5
- FWCI
- 2.12
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- The FASEB Journal (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Digital Commons - East Tennessee State University (East Tennessee State University) (submittedVersion)
- Funders
- National Institutes of Health (CA-86927)
- National Cancer Institute
- Progeria Research Foundation
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Health Sciences › Medicine › Physiology › Telomeres, Telomerase, and Senescence
- Keywords
- XPA, Hutchinson-Gilford progeria syndrome, restrictive dermopathy, premature aging, DNA double-strand break repair, ZMPSTE24, RAD50, RAD51, γH2AX, nucleotide excision repair, DNA repair deficiency, genomic instability
- MeSH terms
- Cells, Cultured, DNA, DNA Damage, DNA Repair, Humans, Nuclear Proteins, Progeria, Protein Binding, Protein Precursors, RNA Interference, Lamin Type A, Xeroderma Pigmentosum Group A Protein
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