Hierarchical deterioration of body systems in Werner's syndrome: Implications for normal ageing
Identifieur interne : 007D12 ( Istex/Curation ); précédent : 007D11; suivant : 007D13Hierarchical deterioration of body systems in Werner's syndrome: Implications for normal ageing
Auteurs : Makoto Goto [Japon]Source :
- Mechanisms of Ageing and Development [ 0047-6374 ] ; 1997.
English descriptors
- KwdEn :
- Adhesion molecules, Ageing, Atrophic skin, Autoimmune diseases, Autosomal recessive inheritance, Bilateral cataracts, Body system disorders, Body systems, Brain atrophy, Broblasts, Case reports, Cataract, Cell subset, Characteristic habitus, Chromosomal instability, Circumscribed hyperkeratosis, Clinical manifestations, Clinical signs, Connective tissue, Cultured cells, Cultured skin, De6elopment, Deterioration, Diabetes mellitus, Different mutations, Doctoral dissertation, Early stage, Elsevier science ireland, Goto, Goto mechanisms, Gray hair, Healthy individuals, Helicase, Hierarchical, Hierarchical deterioration, High homology, Home town, Human ageing, Human diploid cell strains, Hypogonadism, Immune, Immune system, Immune system disorders, Japanese patients, Juvenile cataracts, Light body weight, Major body systems, Major differences, Malignancy, Mutation, Natural ageing, Natural history, Natural killer cell activity, Nervous system, Nice body system, Normal ageing, Normal ageing process, Progeroid, Progeroid syndrome, Recq type, Scleroderma, Sequential appearance, Short stature, Skin changes, Skin sclerosis, Stocky trunk, Subcutaneous tissue, Syndrome, Syndrome gene, System disorders, Thyroid dysfunction, Unique syndrome, Uric acid, Wide variety.
- Teeft :
- Adhesion molecules, Ageing, Atrophic skin, Autoimmune diseases, Autosomal recessive inheritance, Bilateral cataracts, Body system disorders, Body systems, Brain atrophy, Broblasts, Case reports, Cataract, Cell subset, Characteristic habitus, Chromosomal instability, Circumscribed hyperkeratosis, Clinical manifestations, Clinical signs, Connective tissue, Cultured cells, Cultured skin, De6elopment, Deterioration, Diabetes mellitus, Different mutations, Doctoral dissertation, Early stage, Elsevier science ireland, Goto, Goto mechanisms, Gray hair, Healthy individuals, Helicase, Hierarchical, Hierarchical deterioration, High homology, Home town, Human ageing, Human diploid cell strains, Hypogonadism, Immune, Immune system, Immune system disorders, Japanese patients, Juvenile cataracts, Light body weight, Major body systems, Major differences, Malignancy, Mutation, Natural ageing, Natural history, Natural killer cell activity, Nervous system, Nice body system, Normal ageing, Normal ageing process, Progeroid, Progeroid syndrome, Recq type, Scleroderma, Sequential appearance, Short stature, Skin changes, Skin sclerosis, Stocky trunk, Subcutaneous tissue, Syndrome, Syndrome gene, System disorders, Thyroid dysfunction, Unique syndrome, Uric acid, Wide variety.
Abstract
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?'
Url:
DOI: 10.1016/S0047-6374(97)00111-5
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ISTEX:FCD06A906AAE4BCEC06280E6E6F0F6F588A396A3Le document en format XML
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<term>Bilateral cataracts</term>
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<front><div type="abstract" xml:lang="en">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?'</div>
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