http://purl.uniprot.org/citations/20080623 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/20080623 | http://www.w3.org/2000/01/rdf-schema#comment | "Patients with Duchenne muscular dystrophy (DMD) have a progressive dilated cardiomyopathy associated with fatal cardiac arrhythmias. Electrical and functional abnormalities have been attributed to cardiac fibrosis; however, electrical abnormalities may occur in the absence of overt cardiac histopathology. Here we show that structural and functional remodeling of the cardiac sarcoplasmic reticulum (SR) Ca(2+) release channel/ryanodine receptor (RyR2) occurs in the mdx mouse model of DMD. RyR2 from mdx hearts were S-nitrosylated and depleted of calstabin2 (FKBP12.6), resulting in "leaky" RyR2 channels and a diastolic SR Ca(2+) leak. Inhibiting the depletion of calstabin2 from the RyR2 complex with the Ca(2+) channel stabilizer S107 ("rycal") inhibited the SR Ca(2+) leak, inhibited aberrant depolarization in isolated cardiomyocytes, and prevented arrhythmias in vivo. This suggests that diastolic SR Ca(2+) leak via RyR2 due to S-nitrosylation of the channel and calstabin2 depletion from the channel complex likely triggers cardiac arrhythmias. Normalization of the RyR2-mediated diastolic SR Ca(2+) leak prevents fatal sudden cardiac arrhythmias in DMD."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.org/dc/terms/identifier | "doi:10.1073/pnas.0908540107"xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Richard S."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Marks A.R."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Reiken S."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Cassan C."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Lacampagne A."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Fauconnier J."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Matecki S."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/author | "Thireau J."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/date | "2010"xsd:gYear |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/name | "Proc Natl Acad Sci U S A"xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/pages | "1559-1564"xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/title | "Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy."xsd:string |
http://purl.uniprot.org/citations/20080623 | http://purl.uniprot.org/core/volume | "107"xsd:string |
http://purl.uniprot.org/citations/20080623 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/20080623 |
http://purl.uniprot.org/citations/20080623 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/20080623 |
http://purl.uniprot.org/uniprot/P11532#attribution-09E974B4A028EEFE8654FD0ABAD944BC | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/20080623 |
http://purl.uniprot.org/uniprot/P11531#attribution-7B43A1E8358A2EECA8935464ED160CDF | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/20080623 |
http://purl.uniprot.org/uniprot/Q9Z2I2#attribution-D9A4692683CC98E0EB4E8070B3E1DAAD | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/20080623 |
http://purl.uniprot.org/uniprot/E9Q401#attribution-D9A4692683CC98E0EB4E8070B3E1DAAD | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/20080623 |
http://purl.uniprot.org/uniprot/#_A0A023ZT56-mappedCitation-20080623 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/20080623 |
http://purl.uniprot.org/uniprot/#_A0A023ZTV5-mappedCitation-20080623 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/20080623 |
http://purl.uniprot.org/uniprot/#_A0A1Q1NN66-mappedCitation-20080623 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/20080623 |