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Hierarchical deterioration of body systems in Werner's syndrome: Implications for normal ageing
Abstract
Normal human ageing is a complicated biological phenomenon. 'Werner's syndrome (WS)', caused by mutations of RecQ type DNA helicase, has been recognized as a top ranking 'segmental' progeroid syndrome. Patients with WS show a wide variety of clinical and biological manifestations in the four major self-assembly body systems (nervous, immune, connective tissue and endocrine-metabolic systems) similar to normal ageing at an early stage of their life, followed by death at an average age of 46. The sequential appearance of clinical and biological deterioration of the body systems observed in WS suggested that the disorder is more than a segmental progeroid syndrome, analysis of which may shed new light on the question 'Why and how we age?'
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“The co-occurrence of deficient immunity and prematurity of age-related morbidities extends to the progeroid syndromes, in which affected individuals begin aging during the second decade of life [66–68].”
From T-cell aging in rheumatoid arthritis (2014) · cites this paper as [66]
In the citing paper
“WS cells senesce prematurely in culture.”
From DNA Repair, Genome Stability, and Aging (2005) · cites this paper as Goto, 1997
In the citing paper
“The human disease WS represents the best model for premature aging in humans (Goto, 1997”
From DNA Repair, Genome Stability, and Aging (2005) · cites this paper as Goto, 1997
In the citing paper
“WS patients develop premature graying, cataract, loss of subcutaneous fat, skin atrophy, osteoporosis, diabetes, atherosclerosis, and malignancies.”
From DNA Repair, Genome Stability, and Aging (2005) · cites this paper as Goto, 1997
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Metadata
- Authors
- Goto, M.
- Affiliations
- Tokyo Metropolitan Ohtsuka Hospital (JP)
- Corresponding author
- Goto, Makoto
- ORCID
- Goto, Makoto
- Venue
- Mechanisms of Ageing and Development
- Publisher
- Elsevier BV
- Published
- 1997-12
- Volume
- 98
- Issue
- 3
- Pages
- 239-254
- ISSN
- 0047-6374 (ISSN-L)
- OpenAlex
- W1979664563
- Cited by
- 240
- References
- 35
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 7 · 2023: 7 · 2022: 9 · 2021: 3 · 2020: 9 · 2019: 3 · 2018: 7 · 2017: 7 · 2016: 12 · 2015: 10 · 2014: 9 · 2013: 17 · 2012: 7
- FWCI
- 3.76
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Mechanisms of Ageing and Development (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Cancer Institute
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Aging › Genetics, Aging, and Longevity in Model Organisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Telomeres, Telomerase, and Senescence
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
- Keywords
- Werner syndrome, progeroid syndromes, aging, premature aging
- MeSH terms
- Aging, Connective Tissue Diseases, Demography, DNA Helicases, Endocrine System Diseases, Female, Genes, Recessive, Humans, Immune System, Male, Metabolic Diseases, Nervous System, Reference Values, Werner Syndrome
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