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Am J Physiol Cell Physiol (January 10, 2007). doi:10.1152/ajpcell.00295.2006
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Submitted on May 27, 2006
Accepted on January 4, 2007

{beta}-defensin overexpression induces progressive muscle degeneration in mice

Yasuhiro Yamaguchi1, Takahide Nagase2, Tetsuji Tomita1, Kyoko Nakamura3, Shigetomo Fukuhara4, Tomokazu Amano5, Hiroshi Yamamoto6, Yukie Ide7, Misao Suzuki8, Shinji Teramoto9, Tomoichiro Asano5, Kenji Kangawa10, Naomi Nakagata7, Yasuyoshi Ouchi9*, and Hiroki Kurihara5

1 Department of Geriatric Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Bunkyo-ku, Japan
2 Department of Respiratory Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Japan
3 Department of Embryogenesis, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto, Kumamoto, Japan
4 Department of Structural Analysis, National Cardiovascular Center, Suita, Osaka, Japan
5 Department of Physiological Chemistry and Metabolism, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
6 Department of Geriatric Medicine, University of Tokyo, Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan
7 Division of Reproductive Engineering, Center for Animal Resources and Development, Kumamoto University, Kumamoto, Kumamoto, Japan
8 Division of Transgenic Technology,Center for Animal Resources and Development, Kumamoto University, Kumamoto, Kumamoto, Japan
9 Department of Geriatric Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
10 Department of Biochemistry, National Cardiovascular Center Research I, Suita, Osaka, Japan

* To whom correspondence should be addressed. E-mail: youchi-tky{at}umin.ac.jp.

Defensins comprise a family of cationic antimicrobial peptides characterized by the conserved cysteine residues. They are produced in various organs including skeletal muscle, and are identified as key elements in the host defense system as potent effectors. At the same time, defensins have potential roles in the regulation of inflammation and, furthermore, can exert cytotoxic effects on several mammalian cells. Here, we developed transgenic mice overexpressing mouse {beta}-defensin-6 to explore the pathophysiological roles of defensin family as a novel mediator of inflammatory tissue injury. Unexpectedly, the transgenic mice showed short life-span, poor growth, and progressive myofiber degeneration with the functional muscle impairment, predominant centronucleated myofibers and elevated serum creatine kinase activity, as seen in human muscular dystrophy. Furthermore, some of the transgenic myofibers showed I{kappa}B{alpha} accumulation, which would be related to the myofiber apoptosis of Limb-girdle muscular dystrophy type 2A. The present findings may unravel a concealed linkage between the innate immune system and the pathophysiology of degenerative diseases.




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Evaluation of the effect of human {beta}-defensins on neutrophil apoptosis
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[Abstract] [Full Text] [PDF]


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