Partial dataset related to the article: "Fibrosis Rescue Improves Cardiac Function in Dystrophin-Deficient Mice and Duchenne Patient-Specific Cardiomyocytes by Immunoproteasome Modulation".

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<br> characterized by inflammatory cell infiltration, necrosis, and cardiac fibrosis. Standard<br> treatments consider the use of b-blockers and angiotensin-converting enzyme inhibitors that are<br> symptomatic and unspecific toward DMD disease. Medications that target DMD cardiac fibrosis are<br> in the early stages of development. We found immunoproteasome dysregulation in affected hearts<br> of mdx mice (murine animal model of DMD) and cardiomyocytes derived from induced pluripotent<br> stem cells of patients with DMD. Interestingly, immunoproteasome inhibition ameliorated cardiomyopathy<br> in mdx mice and reduced the development of cardiac fibrosis. Establishing the<br> immunoproteasome inhibitionedependent cardioprotective role suggests the possibility of modulating<br> the immunoproteasome as new and clinically relevant treatment to rescue dilated cardiomyopathy<br> in patients with DMD.