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Immunohistochemical study of calpain and its endogenous inhibitor in the skeletal muscle of muscular dystrophy

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Abstract

A calcium-dependent proteinase (calpain) has been suggested to play an important role in muscle degradation in Duchenne muscular dystrophy (DMD). In immunohistochemical studies, calpain and its endogenous inhibitor (calpastatin) were located exclusively in the cytoplasm in normal human muscles. The intensity of the staining was stronger in type 1 than in type 2 fibers. Quantitative immunohistochemical study showed an increase of calpain in biopsied muscles from the patients with DMD and Becker muscular dystrophy. Abnormal increases in calpain and calpastatin were demonstrated mainly in atrophic fibers, whereas necrotic fibers showed moderate or weak immunoreactions for the enzymes. Opaque fibers and hypertrophic fibers were negative. Not all dystrophin-deficient muscle fibers necessarily showed a strong reaction for calpain. We suggest that calpain may play an important role in muscle fiber degradation, especially in the early stage of muscle degradation in muscular dystrophy.

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Authors and Affiliations

  1. Third Department of Internal Medicine, Oita Medical University, 1-1 Hasama, 879-55, Oita, Japan

    Toshihide Kumamoto & Tomiyasu Tsuda

  2. First Department of Internal Medicine, Kumamoto University School of Medicine, Honjo 1-1-1, 860, Kumamoto, Japan

    Hidetsugu Ueyama, Susumu Watanabe & Masayuki Ando

  3. Department of Child Development, Kumamoto University School of Medicine, Honjo 1-1-1, 860, Kumamoto, Japan

    Kowashi Yoshioka & Teruhisa Miike

  4. Muscle Biology Group, University of Arizona, Tucson, Arizona, USA

    Darrel E. Goll

Authors
  1. Toshihide Kumamoto
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  2. Hidetsugu Ueyama
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  3. Susumu Watanabe
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  4. Kowashi Yoshioka
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  5. Teruhisa Miike
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  6. Darrel E. Goll
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  7. Masayuki Ando
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  8. Tomiyasu Tsuda
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Kumamoto, T., Ueyama, H., Watanabe, S. et al. Immunohistochemical study of calpain and its endogenous inhibitor in the skeletal muscle of muscular dystrophy. Acta Neuropathol 89, 399–403 (1995). https://doi.org/10.1007/BF00307642

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  • DOI: https://doi.org/10.1007/BF00307642

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