Genetic Background Affects Properties of Satellite Cells and Mdx Phenotypes

Overview

Journal Am J Pathol

Publisher Elsevier

Specialty Pathology

Date 2010 Mar 23

PMID 20304955

Citations 111

Authors

So-Ichiro Fukada

Daisuke Morikawa

Yukiko Yamamoto

Tokuyuki Yoshida

Noriaki Sumie

Masahiko Yamaguchi

Takahito Ito

Yuko Miyagoe-Suzuki

Shinichi Takeda

Kazutake Tsujikawa

Hiroshi Yamamoto

Affiliations

Soon will be listed here.

Abstract

Duchenne muscular dystrophy (DMD) is the most common lethal genetic disorder of children. The mdx (C57BL/10 background, C57BL/10-mdx) mouse is a widely used model of DMD, but the histopathological hallmarks of DMD, such as the smaller number of myofibers, accumulation of fat and fibrosis, and insufficient regeneration of myofibers, are not observed in adult C57BL/10-mdx except for in the diaphragm. In this study, we showed that DBA/2 mice exhibited decreased muscle weight, as well as lower myofiber numbers after repeated degeneration-regeneration cycles. Furthermore, the self-renewal efficiency of satellite cells of DBA/2 is lower than that of C57BL/6. Therefore, we produced a DBA/2-mdx strain by crossing DBA/2 and C57BL/10-mdx. The hind limb muscles of DBA/2-mdx mice exhibited lower muscle weight, fewer myofibers, and increased fat and fibrosis, in comparison with C57BL/10-mdx. Moreover, remarkable muscle weakness was observed in DBA/2-mdx. These results indicate that the DBA/2-mdx mouse is a more suitable model for DMD studies, and the efficient satellite cell self-renewal ability of C57BL/10-mdx might explain the difference in pathologies between humans and mice.

Citing Articles

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