title: P2Y2purinergic receptors are highly expressed in cardiac and diaphragm muscles of mdx mice, and their expression is decreased by suramin.
abstract: 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.
  
  
  
  In this study, we evaluated whether P2 receptors are involved in cardiomyopathy in mdx mice at later stages of the disease.
  
  
  
  Western blotting revealed that P2Y2receptor levels were upregulated (54%) in dystrophic heart compared with a normal heart. Suramin reduced the levels of P2Y2to almost normal values. Suramin also decreased heart necrosis (reduced CK-MB) and the expression of the stretch-activated calcium channel TRPC1.
  
  
  
  This study suggests that P2Y2may 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.
authors.affiliation: Departamento de Biologia Estrutural e Funcional, Instituto de Biologia, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, 13083-970, Brazil.
authors.forename: Drielen
authors.initials: D
authors.lastname: De Oliveira Moreira
authors.affiliation: Departamento de Biologia Estrutural e Funcional, Instituto de Biologia, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, 13083-970, Brazil.
authors.forename: Humberto
authors.initials: H
authors.lastname: Santo Neto
authors.affiliation: Departamento de Biologia Estrutural e Funcional, Instituto de Biologia, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, 13083-970, Brazil.
authors.forename: Maria Julia
authors.initials: MJ
authors.lastname: Marques
mesh_terms: D000818:Animals; D000969:Antinematodal Agents / Q000494:pharmacology*; D002118:Calcium / Q000378:metabolism; D003402:Creatine Kinase / Q000097:blood; D003964:Diaphragm / Q000187:drug effects* / Q000378:metabolism; D004195:Disease Models, Animal; D005786:Gene Expression Regulation / Q000187:drug effects* / Q000235:genetics; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000473:pathology; D009206:Myocardium / Q000378:metabolism*; D058473:Receptors, Purinergic P2Y2 / Q000235:genetics / Q000378:metabolism*; D013498:Suramin / Q000494:pharmacology*; D050052:TRPC Cation Channels / Q000378:metabolism
chemical_list: D000969:Antinematodal Agents; D058473:Receptors, Purinergic P2Y2; D050052:TRPC Cation Channels; C096351:transient receptor potential cation channel, subfamily C, member 1; D013498:Suramin; D003402:Creatine Kinase; D002118:Calcium
keywords: DMD; P2Y2; cardiomyopathy; mdx; purinergic receptor; suramin