PTX3 Predicts Myocardial Damage and Fibrosis in Duchenne Muscular Dystrophy

PTX3 可预测杜氏肌营养不良症患者的心肌损伤和纤维化

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作者:Andrea Farini ,Chiara Villa ,Dario Di Silvestre ,Pamela Bella ,Luana Tripodi ,Rossana Rossi ,Clementina Sitzia ,Stefano Gatti ,Pierluigi Mauri ,Yvan Torrente

Abstract

Pentraxin 3 (PTX3) is a main component of the innate immune system by inducing complement pathway activation, acting as an inflammatory mediator, coordinating the functions of macrophages/dendritic cells and promoting apoptosis/necrosis. Additionally, it has been found in fibrotic regions co-localizing with collagen. In this work, we wanted to investigate the predictive role of PTX3 in myocardial damage and fibrosis of Duchenne muscular dystrophy (DMD). DMD is an X-linked recessive disease caused by mutations of the dystrophin gene that affects muscular functions and strength and accompanying dilated cardiomyopathy. Here, we expound the correlation of PTX3 cardiac expression with age and Toll-like receptors (TLRs)/interleukin-1 receptor (IL-1R)-MyD88 inflammatory markers and its modulation by the so-called alarmins IL-33, high-mobility group box 1 (HMGB1), and S100β. These findings suggest that cardiac levels of PTX3 might have prognostic value and potential in guiding therapy for DMD cardiomyopathy.

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