caa a22 4500 555159701 CHVBK 20190314032159.0 cr unu---uuuuu 190202e201902 xx s 000 0 eng 10.1016/j.ajpath.2018.10.010 doi (SERVAL)BIB_EF531591F4EE 30448404 pmid 000458713400012 isiid Fibrosis Rescue Improves Cardiac Function in Dystrophin-Deficient Mice and Duchenne Patient-Specific Cardiomyocytes by Immunoproteasome Modulation [Elektronische Daten] [A. Farini, A. Gowran, P. Bella, C. Sitzia, A. Scopece, E. Castiglioni, D. Rovina, P. Nigro, C. Villa, F. Fortunato, G.P. Comi, G. Milano, G. Pompilio, Y. Torrente] 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 β-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 inhibition-dependent cardioprotective role suggests the possibility of modulating the immunoproteasome as new and clinically relevant treatment to rescue dilated cardiomyopathy in patients with DMD. Farini A. aut Gowran A. aut Bella P. aut Sitzia C. aut Scopece A. aut Castiglioni E. aut Rovina D. aut Nigro P. aut Villa C. aut Fortunato F. aut Comi G.P. aut Milano G. aut Pompilio G. aut Torrente Y. aut The American journal of pathology 189/2(2019-02), 339-353 189:2<339-353 2019 189 1 article serval SERVAL 700 1- Farini A. aut SERVAL 700 1- Gowran A. aut SERVAL 700 1- Bella P. aut SERVAL 700 1- Sitzia C. aut SERVAL 700 1- Scopece A. aut SERVAL 700 1- Castiglioni E. aut SERVAL 700 1- Rovina D. aut SERVAL 700 1- Nigro P. aut SERVAL 700 1- Villa C. aut SERVAL 700 1- Fortunato F. aut SERVAL 700 1- Comi G.P. aut SERVAL 700 1- Milano G. aut SERVAL 700 1- Pompilio G. aut SERVAL 700 1- Torrente Y. aut SERVAL 773 0- The American journal of pathology 189/2(2019-02), 339-353 189:2<339-353 2019 189 BK010053 XK010053 XK010000 SERVAL SERVAL SERVAL article