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Muscular dystrophy: Centronucleation may reflect a compensatory activation of defective myonuclei

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Journal of Biomedical Science

Abstract

Muscular dystrophy has long been believed to be characterized by degeneration and abortive regeneration of muscle fibers (the muscle degeneration theory), but unfortunately its pathogenesis is still unclear and an effective treatment has yet to be developed. As a challenge to the theory, we have proposed an alternative muscle-defective-growth theory and a further bone muscle growth imbalance hypothesis supposing possible defects in bone-growth-dependent muscle growth based on our findings in hereditary dystrophic dy mice (C57BL/6Jdy/dy). This review presents some new insights into the pathogenesis of the disease along with our hypothesis, focusing on the physiological meaning of centronucleation, one of the major pathological changes commonly observed in dystrophic muscles of man and experimental animals.

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Authors and Affiliations

  1. Faculty of Pharmaceutical Sciences, Nagoya City University, Nagoya, Japan

    Takaharu Mizutani

  2. Department of Physiology, Aichi Prefectural Institute for Developmental Research, 480-0392, Kasugai, Aichi, Japan

    Tsuyoshi Totsuka PhD, Kimi Watanabe, Isao Uramoto & Kunihiro Sakuma

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  1. Tsuyoshi Totsuka PhD
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Totsuka, T., Watanabe, K., Uramoto, I. et al. Muscular dystrophy: Centronucleation may reflect a compensatory activation of defective myonuclei. J Biomed Sci 5, 54–61 (1998). https://doi.org/10.1007/BF02253356

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