Literature DB >> 8070559

Alpha-dystroglycan deficiency correlates with elevated serum creatine kinase and decreased muscle contraction tension in golden retriever muscular dystrophy.

J M Ervasti1, S L Roberds, R D Anderson, N J Sharp, J N Kornegay, K P Campbell.   

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 alpha-dystroglycan and serum creatine kinase levels and the contraction tension measured for a given peroneus longus muscle.

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Year:  1994        PMID： 8070559     DOI： 10.1016/0014-5793(94)00748-9

Source DB:  PubMed          Journal:  FEBS Lett        ISSN： 0014-5793            Impact factor:   4.124


  7 in total

1.  Muscular dystrophy associated with alpha-dystroglycan deficiency in Sphynx and Devon Rex cats.

Authors:  Paul T Martin; G Diane Shelton; Peter J Dickinson; Beverly K Sturges; Rui Xu; Richard A LeCouteur; Ling T Guo; Robert A Grahn; Harriet P Lo; Kathryn N North; Richard Malik; Eva Engvall; Leslie A Lyons
Journal:  Neuromuscul Disord       Date:  2008-11-05       Impact factor: 4.296

2.  Oral quercetin administration transiently protects respiratory function in dystrophin-deficient mice.

Authors:  Joshua T Selsby; Christopher G Ballmann; Hannah R Spaulding; Jason W Ross; John C Quindry
Journal:  J Physiol       Date:  2016-05-29       Impact factor: 5.182

3.  Dystrophin insufficiency causes selective muscle histopathology and loss of dystrophin-glycoprotein complex assembly in pig skeletal muscle.

Authors:  Katrin Hollinger; Cai X Yang; Robyn E Montz; Dan Nonneman; Jason W Ross; Joshua T Selsby
Journal:  FASEB J       Date:  2013-12-17       Impact factor: 5.191

4.  Sparing of the dystrophin-deficient cranial sartorius muscle is associated with classical and novel hypertrophy pathways in GRMD dogs.

Authors:  Peter P Nghiem; Eric P Hoffman; Priya Mittal; Kristy J Brown; Scott J Schatzberg; Svetlana Ghimbovschi; Zuyi Wang; Joe N Kornegay
Journal:  Am J Pathol       Date:  2013-11       Impact factor: 4.307

5.  Long-Term Quercetin Dietary Enrichment Partially Protects Dystrophic Skeletal Muscle.

Authors:  Hannah R Spaulding; Christopher G Ballmann; John C Quindry; Joshua T Selsby
Journal:  PLoS One       Date:  2016-12-15       Impact factor: 3.240

6.  The effect of the pathological V72I, D109N and T190M missense mutations on the molecular structure of α-dystroglycan.

Authors:  Sonia Covaceuszach; Manuela Bozzi; Maria Giulia Bigotti; Francesca Sciandra; Petr V Konarev; Andrea Brancaccio; Alberto Cassetta
Journal:  PLoS One       Date:  2017-10-16       Impact factor: 3.240

7.  Short-term treatment of golden retriever muscular dystrophy (GRMD) dogs with rAAVrh74.MHCK7.GALGT2 induces muscle glycosylation and utrophin expression but has no significant effect on muscle strength.

Authors:  Paul T Martin; Deborah A Zygmunt; Anna Ashbrook; Sonia Hamilton; Davin Packer; Sharla M Birch; Amanda K Bettis; Cynthia J Balog-Alvarez; Lee-Jae Guo; Peter P Nghiem; Joe N Kornegay
Journal:  PLoS One       Date:  2021-03-26       Impact factor: 3.240
  7 in total

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