Skip to main content

Research Repository

See what's under the surface

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

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

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. 2

Journal Article Type Article
Publication Date Feb 26, 2019
Print ISSN 2045-2322
Publisher Nature Publishing Group
Peer Reviewed Peer Reviewed
Volume 9
Issue 1
Article Number 2770
Institution Citation Omairi, S., Hau, K., Collins-Hooper, H., Scott, C., Vaiyapuri, S., Torelli, S., …Patel, K. (2019). Regulation of the dystrophin-associated glycoprotein complex composition by the metabolic properties of muscle fibres. Scientific reports, 9(1), https://doi.org/10.1038/s41598-019-39532-4
DOI https://doi.org/10.1038/s41598-019-39532-4
Keywords Multidisciplinary
Publisher URL https://www.nature.com/articles/s41598-019-39532-4
Additional Information Received: 29 December 2017; Accepted: 10 January 2019; First Online: 26 February 2019; : The authors declare no competing interests.

Files

Article (8.3 Mb)
PDF

Copyright Statement
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. Te images or other third party material in this
article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
© The Author(s) 2019







Downloadable Citations