{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T22:05:44Z","timestamp":1791324344063,"version":"4.3.1"},"reference-count":181,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2021,2,2]],"date-time":"2021-02-02T00:00:00Z","timestamp":1612224000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Proteomes"],"abstract":"<jats:p>The systematic bioanalytical characterization of the protein product of the DMD gene, which is defective in the pediatric disorder Duchenne muscular dystrophy, led to the discovery of the membrane cytoskeletal protein dystrophin. Its full-length muscle isoform Dp427-M is tightly linked to a sarcolemma-associated complex consisting of dystroglycans, sarcoglyans, sarcospan, dystrobrevins and syntrophins. Besides these core members of the dystrophin\u2013glycoprotein complex, the wider dystrophin-associated network includes key proteins belonging to the intracellular cytoskeleton and microtubular assembly, the basal lamina and extracellular matrix, various plasma membrane proteins and cytosolic components. Here, we review the central role of the dystrophin complex as a master node in muscle fibers that integrates cytoskeletal organization and cellular signaling at the muscle periphery, as well as providing sarcolemmal stabilization and contractile force transmission to the extracellular region. The combination of optimized tissue extraction, subcellular fractionation, advanced protein co-purification strategies, immunoprecipitation, liquid chromatography and two-dimensional gel electrophoresis with modern mass spectrometry-based proteomics has confirmed the composition of the core dystrophin complex at the sarcolemma membrane. Importantly, these biochemical and mass spectrometric surveys have identified additional members of the wider dystrophin network including biglycan, cavin, synemin, desmoglein, tubulin, plakoglobin, cytokeratin and a variety of signaling proteins and ion channels.<\/jats:p>","DOI":"10.3390\/proteomes9010009","type":"journal-article","created":{"date-parts":[[2021,2,2]],"date-time":"2021-02-02T05:44:42Z","timestamp":1612244682000},"page":"9","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["The Dystrophin Node as Integrator of Cytoskeletal Organization, Lateral Force Transmission, Fiber Stability and Cellular Signaling in Skeletal Muscle"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9290-9267","authenticated-orcid":false,"given":"Paul","family":"Dowling","sequence":"first","affiliation":[{"name":"Department of Biology, Maynooth University, National University of Ireland, W23F2H6 Maynooth, Co. Kildare, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, W23F2H6 Maynooth, Co. Kildare, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Gargan","sequence":"additional","affiliation":[{"name":"Department of Biology, Maynooth University, National University of Ireland, W23F2H6 Maynooth, Co. Kildare, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, W23F2H6 Maynooth, Co. Kildare, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1130-9295","authenticated-orcid":false,"given":"Sandra","family":"Murphy","sequence":"additional","affiliation":[{"name":"Newcastle Fibrosis Research Group, Newcastle University Biosciences Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE24HH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margit","family":"Zweyer","sequence":"additional","affiliation":[{"name":"Department of Neonatology and Paediatric Intensive Care, Children\u2019s Hospital, University of Bonn, D53113 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hemmen","family":"Sabir","sequence":"additional","affiliation":[{"name":"Department of Neonatology and Paediatric Intensive Care, Children\u2019s Hospital, University of Bonn, D53113 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0923-7090","authenticated-orcid":false,"given":"Dieter","family":"Swandulla","sequence":"additional","affiliation":[{"name":"Institute of Physiology II, University of Bonn, D53115 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6266-4510","authenticated-orcid":false,"given":"Kay","family":"Ohlendieck","sequence":"additional","affiliation":[{"name":"Department of Biology, Maynooth University, National University of Ireland, W23F2H6 Maynooth, Co. Kildare, Ireland"},{"name":"Kathleen Lonsdale Institute for Human Health Research, Maynooth University, W23F2H6 Maynooth, Co. Kildare, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1016\/0092-8674(87)90504-6","article-title":"Complete cloning of the Duchenne muscular dystrophy (DMD) cDNA and preliminary genomic organization of the DMD gene in normal and affected individuals","volume":"50","author":"Koenig","year":"1987","journal-title":"Cell"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/0092-8674(87)90579-4","article-title":"Dystrophin: The protein product of the Duchenne muscular dystrophy locus","volume":"51","author":"Hoffman","year":"1987","journal-title":"Cell"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1016\/0092-8674(88)90383-2","article-title":"The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein","volume":"53","author":"Koenig","year":"1988","journal-title":"Cell"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1016\/S1474-4422(03)00585-4","article-title":"Dystrophin and mutations: One gene, several proteins, multiple phenotypes","volume":"2","author":"Muntoni","year":"2003","journal-title":"Lancet Neurol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1186\/s13395-016-0095-5","article-title":"Characterization of a Dmd (EGFP) reporter mouse as a tool to investigate dystrophin expression","volume":"6","author":"Petkova","year":"2016","journal-title":"Skelet. 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