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.

PTX3 Predicts Myocardial Damage and Fibrosis in Duchenne Muscular Dystrophy / A. Farini, C. Villa, D. Di Silvestre, P. Bella, L. Tripodi, R. Rossi, C. Sitzia, S. Gatti, P. Mauri, Y. Torrente. - In: FRONTIERS IN PHYSIOLOGY. - ISSN 1664-042X. - 11(2020 May 19). [10.3389/fphys.2020.00403]

PTX3 Predicts Myocardial Damage and Fibrosis in Duchenne Muscular Dystrophy

C. Villa
Data Curation
;
L. Tripodi
Methodology
;
C. Sitzia
Membro del Collaboration Group
;
Y. Torrente
Ultimo
2020-05-19

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.
alarmins; cardiomyopathy; Duchenne muscular dystrophy (DMD); muscular dystrophy; pentraxin 3 (PTX3)
Settore MED/26 - Neurologia
Article (author)
File in questo prodotto:
File Dimensione Formato  
fphys-11-00403.pdf

accesso aperto

Tipologia: Publisher's version/PDF
Dimensione 4.43 MB
Formato Adobe PDF
4.43 MB Adobe PDF Visualizza/Apri
Pubblicazioni consigliate

Caricamento pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/826272
Citazioni
  • ???jsp.display-item.citation.pmc??? 3
  • Scopus 5
  • ???jsp.display-item.citation.isi??? 5
social impact