P2Y(2) purinergic receptors are highly expressed in cardiac and diaphragm muscles of mdx mice, and their expression is decreased by suramin

Author(s)

Moreira, Drielen De Oliveira ; Neto, Humberto Santo ; Marques, Maria Julia

• Document type: Journal article
• Source: MUSCLE & NERVE; v. 55, n. 1, p. 116-121, JAN 2017.
• Web of Science Citations: 3

Abstract

Introduction: In Duchenne muscular dystrophy (DMD) and in the mdx mouse model of DMD, the lack of dystrophin leads to increased calcium influx and muscle necrosis. Patients suffer progressive muscle loss, and cardiomyopathy is an important determinant of morbidity. P2 purinergic receptors participate in the increased calcium levels in dystrophic skeletal muscles. Methods: In this study, we evaluated whether P2 receptors are involved in cardiomyopathy in mdx mice at later stages of the disease. Results: Western blotting revealed that P2Y(2) receptor levels were upregulated (54%) in dystrophic heart compared with a normal heart. Suramin reduced the levels of P2Y(2) to almost normal values. Suramin also decreased heart necrosis (reduced CK-MB) and the expression of the stretch-activated calcium channel TRPC1. Conclusions: This study suggests that P2Y(2) may participate in cardiomyopathy in mdx mice. P2-selective drugs with specific actions in the dystrophic heart may ameliorate cardiomyopathy in dystrophinopathies. Muscle Nerve55: 116-121, 2017 (AU)

• FAPESP's process: 12/03498-7 - Mechanistic insights on the effects of suramin, on the cardiomyopathy of the mdx mice.
• Grantee: Drielen de Oliveira Moreira
• Support type: Scholarships in Brazil - Doctorate
• FAPESP's process: 14/04782-6 - Pre-clinical studies in the mdx mouse: metabolomics, biomarkers and omega-3 therapy
• Grantee: Maria Julia Marques
• Support type: Regular Research Grants