Cited by 304Nuclear Structure and FunctionTelomeres, Telomerase, and SenescenceGenetics, Aging, and Longevity in Model Organisms

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Werner and Hutchinson–Gilford progeria syndromes: mechanistic basis of human progeroid diseases

Kudlow, Brian A., Kennedy, Brian K., Monnat, Raymond J.·2007·Nature Reviews Molecular Cell Biology

DOI 10.1038/nrm2161

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Abstract

Progeroid syndromes have been the focus of intense research in part because they might provide a window into the pathology of normal ageing. Werner syndrome and Hutchinson-Gilford progeria syndrome are two of the best characterized human progeroid diseases. Mutated genes that are associated with these syndromes have been identified, mouse models of disease have been developed, and molecular studies have implicated decreased cell proliferation and altered DNA-damage responses as common causal mechanisms in the pathogenesis of both diseases.

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Authors: Kudlow, B. A., Kennedy, B. K., & Monnat, R. J.
Affiliations: University of Washington (US)
Corresponding author: Kennedy, Brian K.
Monnat, Raymond J.
ORCID: Kennedy, Brian K.
Monnat, Raymond J.
Venue: Nature Reviews Molecular Cell Biology
Publisher: Springer Science and Business Media LLC
Published: 2007-05
Volume: 8
Issue: 5
Pages: 394-404
ISSN: 1471-0072 (ISSN-L)
1471-0080
DOI: 10.1038/nrm2161
OpenAlex: W2163819508
Cited by: 304
References: 110
Citations by year: 2026: 10 · 2025: 8 · 2024: 12 · 2023: 12 · 2022: 17 · 2021: 11 · 2020: 14 · 2019: 13 · 2018: 18 · 2017: 12 · 2016: 17 · 2015: 14 · 2014: 10 · 2013: 14 · 2012: 14
FWCI: 2.28
Type: journal article
Language: en
Open access: closed
Licence: www.springer.com/tdm (tdm)
Locations: Nature Reviews Molecular Cell Biology (publishedVersion)
PubMed (publishedVersion)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Telomeres, Telomerase, and Senescence
Life Sciences › Biochemistry, Genetics and Molecular Biology › Aging › Genetics, Aging, and Longevity in Model Organisms
Keywords: Werner syndrome, Hutchinson-Gilford progeria syndrome, progeroid syndromes, premature aging, DNA damage response, cell proliferation, mouse models of human disease, genetic mutations, normal aging
MeSH terms: Werner Syndrome Helicase, Aging, Animals, Disease Models, Animal, DNA Damage, Exodeoxyribonucleases, Humans, Models, Biological, Phenotype, Progeria, Protein Processing, Post-Translational, Werner Syndrome, Lamin Type A, RecQ Helicases

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Werner and Hutchinson–Gilford progeria syndromes: mechanistic basis of human progeroid diseases

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