Cited by 240Genetics, Aging, and Longevity in Model OrganismsTelomeres, Telomerase, and SenescenceNuclear Structure and Function

Hierarchical deterioration of body systems in Werner's syndrome: Implications for normal ageing

Goto, Makoto·1997·Mechanisms of Ageing and Development

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?'

Integrity signals

Registry

Crossref · 10.1016/s0047-6374(97)00111-5

Cites

34 of 34 references verified0 citation errors · 0 not checkable

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  • Progeroid syndromes, in which affected individuals begin aging during the second decade of life, share the co-occurrence of deficient immunity and premature age-related morbidities.

    “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]

  • WS cells senesce prematurely in culture.

    “WS cells senesce prematurely in culture.”

    From DNA Repair, Genome Stability, and Aging (2005) · cites this paper as Goto, 1997

  • The human disease WS represents the best model for premature aging in humans.

    “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

  • WS patients develop premature graying, cataract, loss of subcutaneous fat, skin atrophy, osteoporosis, diabetes, atherosclerosis, and malignancies.

    “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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Authors
Goto, M.
Affiliations
Tokyo Metropolitan Ohtsuka Hospital (JP)
Corresponding author
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
  • Health Sciences › Medicine › Physiology › 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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