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Research article| Volume 119, ISSUE 1, P43-52, October 1993

Reciprocal expression of dystrophin and utrophin in muscles of Duchenne muscular dystrophy patients, female DMD-carriers and control subjects

  • Yuji Mizuno
    Correspondence
    Correspondence to: Yuji Mizuno, M.D., National Institute of Neuroscience, NCNP, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187, Japan. Tel.: (81)-423-41-2711; Fax: (81)-423-46-1750.
    Affiliations
    National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Tokyo, Japan

    Department of Neurology, Gunma University, Maebashi, Gunma, Japan
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  • Ikuya Nonaka
    Affiliations
    National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Tokyo, Japan
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  • Shunsaku Hirai
    Affiliations
    Department of Neurology, Gunma University, Maebashi, Gunma, Japan
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  • Eijiro Ozawa
    Affiliations
    National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Tokyo, Japan
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DOI:https://doi.org/10.1016/0022-510X(93)90190-A
Reciprocal expression of dystrophin and utrophin in muscles of Duchenne muscular dystrophy patients, female DMD-carriers and control subjects
Previous ArticleIntrafamilial variability in dystrophin abundance correlated with difference in the severity of the phenotype
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      Abstract

      We examined muscle biopsies from patients with Duchenne muscular dystrophy (DMD: 39 patients) and Becker muscular dystrophy (BMD: 11 patients), female DMD-carriers (4 patients), and control subjects (26 persons) for the expression of dystrophin and utrophin. Control subjects showed all fibers to be dystrophin-positive, while utrophin staining was negative or weak. On the other hand, muscles from DMD patients showed the inverse staining patterns: dystrophin was negative and utrophin staining strong. Thus, there was a reciprocal pattern of expression between dystrophin and utrophin. This reciprocal relationship was confirmed to some extent at the single-fiber level in female carriers of DMD showing a mosaic immunostaining of dystrophin. We consider that utrophin may have a function similar to that of dystrophin, and compensate to some extent for dystrophin deficiency in DMD.

      Keywords

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      Article info

      Publication history

      Accepted: April 15, 1993
      Received in revised form: March 29, 1993
      Received: December 11, 1992

      Identification

      DOI: https://doi.org/10.1016/0022-510X(93)90190-A

      Copyright

      © 1993 Published by Elsevier Inc.

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