Journal of Muscle Research & Cell Motility

, Volume 20, Issue 3, pp 305–314 | Cite as

Utrophin and dystrophin-associated glycoproteins in normal and dystrophin deficient cardiac muscle

  • François Rivier
  • Agnès Robert
  • Mar Royuela
  • Gérald Hugon
  • Armelle Bonet-Kerrache
  • Dominique Mornet


In this study, various members of the dystrophin family (dystrophin, the short dystrophin product Dp 71, utrophin and DRP2), and different members of the dystrophin-associated glycoprotein (DAG) complex (beta-dystroglycan, alpha-, beta-, gamma- and delta-sarcoglycans) were localized in bovine cardiac muscle using a battery of specific antibodies. We have established that dystrophin is exclusively associated with beta-dystroglycan and both alpha- and delta-sarcoglycans in cardiac muscle cell membranes. In contrast, utrophin is a specific component of intercalated disks together with beta- and gamma-sarcoglycans, while beta-dystroglycan, alpha- and delta-sarcoglycans are not present. Dp 71 is mainly localized at the T tubule transverse area. In dystrophin deficient cardiac muscle, utrophin and beta-sarcoglycan were observed in intercalated disks and at the sarcolemma of each cardiocyte. Our results revealed that complexes of associated glycoproteins differ in cardiac muscle when associated with dystrophin or utrophin. Despite the described sequence homologies between dystrophin and utrophin, the present results indicate that these proteins have different roles in some specific cardiac cell areas.


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Copyright information

© Kluwer Academic Publishers 1999

Authors and Affiliations

  • François Rivier
    • 1
  • Agnès Robert
    • 1
  • Mar Royuela
    • 2
  • Gérald Hugon
    • 1
  • Armelle Bonet-Kerrache
    • 1
  • Dominique Mornet
    • 1
  1. 1.Muscles et PathologiesINSERM U128, IFR 24Montpellier, Cedex 5France
  2. 2.Department of Cell Biology and GeneticsUniversity of AlcalàAlcalà de Henares (Madrid)Spain

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