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. 2017 Jul 19;9(7):1738-1744.
doi: 10.18632/aging.101265.

Werner syndrome: a model for sarcopenia due to accelerated aging

Masaya Yamaga  1   2 , Minoru Takemoto  1   3 , Mayumi Shoji  1   2 , Kenichi Sakamoto  1   4 , Masashi Yamamoto  1   2 , Takahiro Ishikawa  1   2 , Masaya Koshizaka  1   2 , Yoshiro Maezawa  1   2 , Kazuki Kobayashi  1   5 , Koutaro Yokote  1   2
Affiliations

Werner syndrome: a model for sarcopenia due to accelerated aging

Masaya Yamaga et al. Aging (Albany NY). .

Abstract

Werner syndrome (WS) is a rare inheritable progeroid syndrome caused by a mutation in the WRN gene. Although WS has been described as a characteristic appearance of very slender extremities with a stocky trunk, few studies have investigated the loss of muscle mass, fat mass distribution (body composition), and mobility according to age and sex. Therefore, the aim of this study was to precisely describe the body composition in WS. Nine Japanese patients with WS (four males and five females; mean age 48±8.8 years) were recruited. Body composition was examined by dual-energy X-ray absorptiometry and computed tomography (CT). The hand grip strength and mobility were evaluated using the two-step test, stand-up test and 25-question geriatric locomotive function scale (GLFS). The mean skeletal muscle index (SMI) was 4.0±0.6 kg/m2. SMI of all patients met the criteria of sarcopenia, even though some patients were aged < 40 years. All patients also showed deceased mobility. In conclusion, these results indicate that all patients with WS, even those aged < 40 years, had already lost muscle mass to the level of sarcopenia. Continued research on sarcopenia in WS might facilitate the discovery of novel mechanisms and development of new treatment strategies for sarcopenia.

Keywords: Werner syndrome; progeria; sarcopenia.

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Conflict of interest statement

CONFLICTS OF INTEREST

All authors have no conflict of interests.

Figures

Figure 1
Figure 1. Correlations among skeletal muscle index (SMI), visceral fat area (VFA), and age
Values for SMI and VFA were plotted against age. SMI is indicated by open circles and VFA is indicated by closed circles. Each line and the dotted line indicate an approximate curve.
See this image and copyright information in PMC

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