Partial dataset related to the article: "Fibrosis Rescue Improves Cardiac Function in Dystrophin-Deficient Mice and Duchenne Patient-Specific Cardiomyocytes by Immunoproteasome Modulation"
| Main Authors: | Andrea Farini, Aoife Gowran, Pamela Bella, Clementina Sitzia, Alessandro Scopece, Elisa Castiglioni, Davide Rovina, Patrizia Nigro, Chiara Villa, Francesco Fortunato, Giacomo Pietro Comi, Giuseppina Milano, Giulio Pompilio |
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| Format: | info dataset |
| Terbitan: |
, 2020
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| Subjects: | |
| Online Access: |
https://zenodo.org/record/3707832 |
Daftar Isi:
- 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.
- Supported by Italian Ministry of Health Conte Capitale 2015 (G.P.), Fondazione Umberto Veronesi (A.G.), and Fondazione IEO CCM (D.R.).