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

doi:10.1016/0014-5793(94)00748-9

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

Biochemistry, Molecular Biology, and Biophysics (TMED)

Research output: Contribution to journal › Article › peer-review

13 Scopus citations

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 language	English (US)
Pages (from-to)	173-176
Number of pages	4
Journal	FEBS Letters
Volume	350
Issue number	2-3
DOIs	https://doi.org/10.1016/0014-5793(94)00748-9
State	Published - 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

Publisher link

10.1016/0014-5793(94)00748-9

Find It

Find It at U of M Libraries

Cite this

@article{22c179e3f8814b8bbb0faf193bc15776,

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

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.",

keywords = "Dystroglycan, Dystrophin, Dystrophin-associated protein, Dystrophin-glycoprotein complex, Golden retriever muscular dystrophy",

author = "Ervasti, {James M.} and Roberds, {Steven L.} and Anderson, {Richard D.} and Sharp, {Nicholas J.H.} and Kornegay, {Joe N.} and Campbell, {Kevin P.}",

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.).",

year = "1994",

month = aug,

day = "22",

doi = "10.1016/0014-5793(94)00748-9",

language = "English (US)",

volume = "350",

pages = "173--176",

journal = "FEBS Letters",

issn = "0014-5793",

publisher = "John Wiley and Sons Inc.",

number = "2-3",

}

TY - JOUR

T1 - α-Dystroglycan deficiency correlates with elevated serum creatine kinase and decreased muscle contraction tension in golden retriever muscular dystrophy

AU - Ervasti, James M.

AU - Roberds, Steven L.

AU - Anderson, Richard D.

AU - Sharp, Nicholas J.H.

AU - Kornegay, Joe N.

AU - Campbell, Kevin P.

N1 - 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.).

PY - 1994/8/22

Y1 - 1994/8/22

N2 - 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.

AB - 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.

KW - Dystroglycan

KW - Dystrophin

KW - Dystrophin-associated protein

KW - Dystrophin-glycoprotein complex

KW - Golden retriever muscular dystrophy

UR - http://www.scopus.com/inward/record.url?scp=0027994474&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0027994474&partnerID=8YFLogxK

U2 - 10.1016/0014-5793(94)00748-9

DO - 10.1016/0014-5793(94)00748-9

M3 - Article

C2 - 8070559

AN - SCOPUS:0027994474

SN - 0014-5793

VL - 350

SP - 173

EP - 176

JO - FEBS Letters

JF - FEBS Letters

IS - 2-3

ER -

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

Abstract

Bibliographical note

Keywords

Publisher link

Find It

Other files and links

Fingerprint

Cite this