Dowling, Paul and Gargan, Stephen and Zweyer, Margit and Sabir, Hemmen and Henry, Michael and Meleady, Paula and Swandulla, Dieter and Ohlendieck, Kay (2021) Proteomic profiling of the interface between the stomach wall and the pancreas in dystrophinopathy. European Journal of Translational Myology, 31 (1). ISSN 2037-7452

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Abstract

The neuromuscular disorder Duchenne muscular dystrophy is a multi-systemic disease that is caused by a primary abnormality in the X-chromosomal Dmd gene. Although progressive skeletal muscle wasting and cardio-respiratory complications are the most serious symptoms that are directly linked to the almost complete loss of the membrane cytoskeletal protein dystrophin, dystrophic patients also suffer from gastrointestinal dysfunction. In order to determine whether proteome-wide changes potentially occur in the gastrointestinal system due to dystrophin deficiency, total tissue extracts from the interface between the stomach wall and the pancreas of the mdx-4cv model of dystrophinopathy were analysed by mass spectrometry. Following the proteomic establishment of both smooth muscle markers of the gastrointestinal system and key enzymes of the pancreas, core members of the dystrophin-glycoprotein complex, including dystrophin, dystroglycans, sarcoglycans, dystrobrevins and syntrophins were identified in this tissue preparation. Comparative proteomics revealed a drastic reduction in dystrophin, sarcoglycan, dystroglycan, laminin, titin and filamin suggesting loss of cytoskeletal integrity in mdx-4cv smooth muscles. A concomitant increase in various mitochondrial enzymesisindicative of metabolic disturbances. These findings agree with abnormal gastrointestinal function in dystrophinopathy

Item Type:	Article
Additional Information:	Cite as: Dowling, P., Gargan, S., Zweyer, M., Sabir, H., Henry, M., Meleady, P., Swandulla, D., & Ohlendieck, K. (2021). Proteomic profiling of the interface between the stomach wall and the pancreas in dystrophinopathy. European Journal of Translational Myology, 31(1). https://doi.org/10.4081/ejtm.2021.9627
Keywords:	Duchenne muscular dystrophy; mdx-4cv; pancreas; proteomics; stomach;
Academic Unit:	Faculty of Science and Engineering > Biology Faculty of Science and Engineering > Research Institutes > Human Health Institute
Item ID:	17268
Identification Number:	https://doi.org/10.4081/ejtm.2021.9627
Depositing User:	Paul Dowling
Date Deposited:	01 Jun 2023 11:24
Journal or Publication Title:	European Journal of Translational Myology
Publisher:	Page Press Journals
Refereed:	Yes
URI:	Publisher
Use Licence:	This item is available under a Creative Commons Attribution Non Commercial Share Alike Licence (CC BY-NC-SA). Details of this licence are available here

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