Regulation of the dystrophin-associated glycoprotein complex composition by the metabolic properties of muscle fibres View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2019-12

AUTHORS

Saleh Omairi, Kwan-Leong Hau, Henry Collins-Hooper, Charlotte Scott, Sakthivel Vaiyapuri, Silvia Torelli, Federica Montanaro, Antonios Matsakas, Ketan Patel

ABSTRACT

The dystrophin-glycoprotein complex (DGC) links the muscle cytoskeleton to the extracellular matrix and is responsible for force transduction and protects the muscle fibres from contraction induced damage. Mutations in components of the DGC are responsible for muscular dystrophies and congenital myopathies. Expression of DGC components have been shown to be altered in many myopathies. In contrast we have very little evidence of whether adaptive changes in muscle impact on DGC expression. In this study we investigated connection between muscle fibre phenotype and the DGC. Our study reveals that the levels of DGC proteins at the sarcolemma differ in highly glycolytic muscle compared to wild-type and that these changes can be normalised by the super-imposition of an oxidative metabolic programme. Importantly we show that the metabolic properties of the muscle do not impact on the total amount of DGC components at the protein level. Our work shows that the metabolic property of a muscle fibre is a key factor in regulating the expression of DGC proteins at the sarcolemma. More... »

PAGES

2770

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/s41598-019-39532-4

DOI

http://dx.doi.org/10.1038/s41598-019-39532-4

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1112393185

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30808964


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