Abstract
Many data pertaining to the accelerated telomere loss in cultured cells derived from Werner syndrome (WS), a representative premature aging syndrome, have been accumulated. However, there have been no definitive data on in vivo telomere shortening in WS patients. In the present study, we measured terminal restriction fragment (TRF) lengths of 10 skin samples collected from extremities of 8 WS patients aged between 30 and 61 years that had been surgically amputated because of skin ulceration, and estimated the annual telomere loss. Whereas the values of TRF length in younger WS patients (in their thirties) were within the normal range, those in older WS patients were markedly shorter relative to non‐WS controls. Regression analyses indicated that the TRF length in WS was significantly shorter than that in controls (p < 0.001). Furthermore, we found that TRF lengths in muscle adjacent to the examined epidermis were also significantly shorter than those of controls (p = 0.047). These data demonstrate for the first time that in vivo telomere loss is accelerated in systemic organs of WS patients, suggesting that abnormal telomere erosion is one of the major causes of early onset of age‐related symptoms and a predisposition to sarcoma and carcinoma in WS.
Highlights
Werner syndrome (WS) is an autosomal recessive disorder characterized by premature onset and an accelerated rate of development of major geriatric diseases, including atherosclerosis, diabetes mellitus, osteoporosis, cataract and menopause, and predisposition to sarcoma and carcinoma [1]
We found that terminal restriction fragment (TRF) lengths in muscle adjacent to the examined epidermis were significantly shorter than those of controls (p = 0.047)
These data demonstrate for the first time that in vivo telomere loss is accelerated in systemic organs of WS patients, suggesting that abnormal telomere erosion is one of the major causes of early onset of age‐related symptoms and a predisposition to sarcoma and carcinoma in WS
Summary
Werner syndrome (WS) is an autosomal recessive disorder characterized by premature onset and an accelerated rate of development of major geriatric diseases, including atherosclerosis, diabetes mellitus, osteoporosis, cataract and menopause, and predisposition to sarcoma and carcinoma [1]. The characteristics that define WS among several other premature aging syndromes are that affected individuals show normal growth and development until adolescence, after which the various symptoms appear segmentally in an individual-specific manner [2]. Human somatic cells are known to have a limited proliferative life span (the Hayflick limit) when serially cultured in vitro [5, 6]. When they approach this limit, they cease to replicate and exhibit a state of replicative senescence.
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