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P2Y 2 purinergic receptors are highly expressed in cardiac and diaphragm muscles of mdx mice, and their expression is decreased by suramin
Author(s) -
De Oliveira Moreira Drielen,
Santo Neto Humberto,
Marques Maria Julia
Publication year - 2017
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25199
Subject(s) - suramin , purinergic receptor , medicine , endocrinology , duchenne muscular dystrophy , dystrophin , cardiomyopathy , mdx mouse , receptor , myocyte , skeletal muscle , calcium , heart failure
: 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 Nerve 55 : 116–121, 2017