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
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.