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Research Paper|Volume 9, Issue 7|pp 1738—1744

Werner syndrome: a model for sarcopenia due to accelerated aging

Masaya Yamaga1,2, Minoru Takemoto1,3, Mayumi Shoji1,2, Kenichi Sakamoto1,4, Masashi Yamamoto1,2, Takahiro Ishikawa1,2, Masaya Koshizaka1,2, Yoshiro Maezawa1,2, Kazuki Kobayashi1,5, Koutaro Yokote1,2
  • 1Department of Clinical Cell Biology and Medicine, Chiba University Graduate School of Medicine, Chiba, 260-8670, Japan
  • 2Department of Medicine, Division of Diabetes, Metabolism and Endocrinology, Chiba University Hospital, Chiba, 260-8670, Japan
  • 3School of Medicine, International University of Health and Welfare, Department of Diabetes, Metabolism and Endocrinology, Chiba, 286-8686, Japan
  • 4Eastern Chiba Medical Center, Chiba, 283-8686, Japan
  • 5Asahi General Hospital, 1326 I, Chiba, 289-2511, Japan
Received: June 16, 2017Accepted: July 19, 2017Published: July 19, 2017
https://doi.org/10.18632/aging.101265

Copyright: © 2017 Yamaga et al. This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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.