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In pre-clinical studies, \u03bcDys efficiently rescues cardiac histopathology, but only partially normalizes cardiac function. To gain insights into factors that may impact the cardiac therapeutic efficacy of \u03bcDys, we compared by mass spectrometry the composition of purified dystrophin and \u03bcDys protein complexes in the mouse heart. We report that compared to dystrophin, \u03bcDys has altered associations with \u03b11- and \u03b22-syntrophins, as well as cavins, a group of caveolae-associated signaling proteins. In particular, we found that membrane localization of cavin-1 and cavin-4 in cardiomyocytes requires dystrophin and is profoundly disrupted in the heart of mdx5cv mice, a model of DMD. Following cardiac stress\/damage, membrane-associated cavin-4 recruits the signaling molecule ERK to caveolae, which activates key cardio-protective responses. Evaluation of ERK signaling revealed a profound inhibition, below physiological baseline, in the mdx5cv mouse heart. Expression of \u03bcDys in mdx5cv mice prevented the development of cardiac histopathology but did not rescue membrane localization of cavins nor did it normalize ERK signaling. Our study provides the first comparative analysis of purified protein complexes assembled in vivo by full-length dystrophin and a therapeutic micro-dystrophin construct. This has revealed disruptions in cavins and ERK signaling that may contribute to DMD cardiomyopathy. This new knowledge is important for ongoing efforts to prevent and treat heart disease in DMD patients.<\/jats:p>","DOI":"10.1093\/hmg\/ddab133","type":"journal-article","created":{"date-parts":[[2021,4,30]],"date-time":"2021-04-30T19:14:50Z","timestamp":1619810090000},"page":"1321-1336","source":"Crossref","is-referenced-by-count":13,"title":["Proteomic analysis identifies key differences in the cardiac interactomes of dystrophin and micro-dystrophin"],"prefix":"10.1093","volume":"30","author":[{"given":"Hong","family":"Wang","sequence":"first","affiliation":[{"name":"Center for Gene Therapy, The Research Institute at Nationwide Children\u2019s Hospital, Columbus OH 43205, USA"},{"name":"Department of Pediatric Cardiology, China Medical University, Liaoning 110004, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Marrosu","sequence":"additional","affiliation":[{"name":"Developmental Neuroscience Research and Teaching Department, Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"NIHR Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Brayson","sequence":"additional","affiliation":[{"name":"Developmental Neuroscience Research and Teaching Department, Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"NIHR Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nalinda B","family":"Wasala","sequence":"additional","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, School of Medicine, University of Missouri, Columbia, MO 65211, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric K","family":"Johnson","sequence":"additional","affiliation":[{"name":"Center for Gene Therapy, The Research Institute at Nationwide Children\u2019s Hospital, Columbus OH 43205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte S","family":"Scott","sequence":"additional","affiliation":[{"name":"Developmental Neuroscience Research and Teaching Department, Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"NIHR Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongping","family":"Yue","sequence":"additional","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, School of Medicine, University of Missouri, Columbia, MO 65211, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kwan-Leong","family":"Hau","sequence":"additional","affiliation":[{"name":"Developmental Neuroscience Research and Teaching Department, Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"NIHR Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aaron J","family":"Trask","sequence":"additional","affiliation":[{"name":"Center for Cardiovascular Research, The Research Institute at Nationwide Children's Hospital, Columbus, OH 43205, USA"},{"name":"Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH 43205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stan C","family":"Froehner","sequence":"additional","affiliation":[{"name":"Department of Physiology and Biophysics, University of Washington, Seattle, WA 98195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marvin E","family":"Adams","sequence":"additional","affiliation":[{"name":"Department of Physiology and Biophysics, University of Washington, Seattle, WA 98195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liwen","family":"Zhang","sequence":"additional","affiliation":[{"name":"Mass Spectrometry and Proteomics Facility, Campus Chemical Instrument Center, The Ohio State University, Columbus, OH 43210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dongsheng","family":"Duan","sequence":"additional","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, School of Medicine, University of Missouri, Columbia, MO 65211, USA"},{"name":"Department of Neurology, School of Medicine, College of Veterinary Medicine, University of Missouri, Columbia, MO 65211, USA"},{"name":"Department of Bioengineering, College of Veterinary Medicine, University of Missouri, Columbia, MO 65211, USA"},{"name":"Department of Biomedical Sciences, College of Veterinary Medicine, University of Missouri, Columbia, MO 65211, USA"},{"name":"Department of Biomedical, Biological and Chemical Engineering, College of Engineering, University of Missouri, Columbia, MO 65211, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Montanaro","sequence":"additional","affiliation":[{"name":"Center for Gene Therapy, The Research Institute at Nationwide Children\u2019s Hospital, Columbus OH 43205, USA"},{"name":"Developmental Neuroscience Research and Teaching Department, Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK"},{"name":"NIHR Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, 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