Partial dataset related to the article: "Fibrosis Rescue Improves Cardiac Function in Dystrophin-Deficient Mice and Duchenne Patient-Specific Cardiomyocytes by Immunoproteasome Modulation".
- 1. Stem Cell Laboratory, Department of Pathophysiology and Transplantation, Universitá degli Studi di Milano, Unit of Neurology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Centro Dino Ferrari, Milan, Italy.
- 2. Unit of Vascular Biology and Regenerative Medicine, Centro Cardiologico Monzino-IRCCS, Milan, Italy.
- 3. UOC SMEL-1, Scuola di Specializzazione di Patologia Clinica e Biochimica Clinica, Università degli Studi di Milano, Milan, Italy.
- 4. Neurology Unit, Neuroscience Section, Department of Pathophysiology and Transplantation, Dino Ferrari Centre, IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
- 5. Laboratory of Cardiovascular Research, Depart of Surgery and Anesthesiology, University Hospital of Lausanne, Lausanne, CH; Unit of Vascular Biology and Regenerative Medicine, Centro Cardiologico Monzino, Italy.
- 6. Department of Cardiac Surgery and Unit of Vascular Biology and Regenerative Medicine, Centro Cardiologico Monzino-IRCCS, Milan, Italy; Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
Description
This record contains raw data related to the article: "Fibrosis Rescue Improves Cardiac Function in Dystrophin-Deficient Mice and Duchenne Patient-Specific Cardiomyocytes by Immunoproteasome Modulation".
Abstract
Patients affected by Duchenne muscular dystrophy (DMD) develop a progressive dilated cardiomyopathy
characterized by inflammatory cell infiltration, necrosis, and cardiac fibrosis. Standard
treatments consider the use of b-blockers and angiotensin-converting enzyme inhibitors that are
symptomatic and unspecific toward DMD disease. Medications that target DMD cardiac fibrosis are
in the early stages of development. We found immunoproteasome dysregulation in affected hearts
of mdx mice (murine animal model of DMD) and cardiomyocytes derived from induced pluripotent
stem cells of patients with DMD. Interestingly, immunoproteasome inhibition ameliorated cardiomyopathy
in mdx mice and reduced the development of cardiac fibrosis. Establishing the
immunoproteasome inhibitionedependent cardioprotective role suggests the possibility of modulating
the immunoproteasome as new and clinically relevant treatment to rescue dilated cardiomyopathy
in patients with DMD.
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- Is derived from
- Journal article: 10.1016/j.ajpath.2018.10.010 (DOI)