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α-Dystroglycan deficiency correlates with elevated serum creatine kinase and decreased muscle contraction tension in golden retriever muscular dystrophy

  • James M. Ervasti
  • , Steven L. Roberds
  • , Richard D. Anderson
  • , Nicholas J.H. Sharp
  • , Joe N. Kornegay
  • , Kevin P. Campbell

Research output: Contribution to journalArticlepeer-review

Abstract

The dystrophin-glycoprotein complex was examined in dystrophin-deficient dogs with golden retriever muscular dystrophy (GRMD) using immunoblot and immunofluorescence analysis. The dystrophin-associated proteins were substantially reduced in muscle from dogs with GRMD. Interestingly, regression analysis revealed a strong correlation between the amount of α-dystroglycan and serum creatine kinase levels and the contraction tension measured for a given peroneus longus muscle.

Original languageEnglish (US)
Pages (from-to)173-176
Number of pages4
JournalFEBS Letters
Volume350
Issue number2-3
DOIs
StatePublished - Aug 22 1994

Bibliographical note

Funding Information:
Acknowledgements: We gratefully acknowledgeD avid Zoloto and Michael Mullinix for preparationo f the skeletaml uscleh omogenates and membranesu sed in this study. K.P.C. is an investigatoro f the Howard HughesM edical Institute.T his work was also supportedb y grants from the Muscular Dystrophy Association (J.M.E., S.L.R., J.N.K. and K.P.C.) and the WisconsinA ffiliate of the AmericanH eart Association( J.M.E.).

Keywords

  • Dystroglycan
  • Dystrophin
  • Dystrophin-associated protein
  • Dystrophin-glycoprotein complex
  • Golden retriever muscular dystrophy

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