Sodium current deficit and arrhythmogenesis in a murine model of plakophilin-2 haploinsufficiency | Zendy

Marina Cerrone | Zendy; Maartje Noorman | Zendy; Xianming Lin | Zendy; Halina Chkourko | Zendy; FengXia Liang | Zendy; Roel van der Nagel | Zendy; Thomas J. Hund | Zendy; Walter Birchmeier | Zendy; Peter J. Mohler | Zendy; Toon A van Veen | Zendy; Harold V.M. van Rijen | Zendy; Mario Delmar | Zendy

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Sodium current deficit and arrhythmogenesis in a murine model of plakophilin-2 haploinsufficiency

Author(s) -

Marina Cerrone,

Maartje Noorman,

Xianming Lin,

Halina Chkourko,

FengXia Liang,

Roel van der Nagel,

Thomas J. Hund,

Walter Birchmeier,

Peter J. Mohler,

Toon A van Veen,

Harold V.M. van Rijen,

Mario Delmar

Publication year - 2012

Publication title -

cardiovascular research

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.774

H-Index - 219

eISSN - 1755-3245

pISSN - 0008-6363

DOI - 10.1093/cvr/cvs218

Subject(s) - haploinsufficiency , medicine , cardiology , chemistry , phenotype , gene , biochemistry

The shRNA-mediated loss of expression of the desmosomal protein plakophilin-2 leads to sodium current (I(Na)) dysfunction. Whether pkp2 gene haploinsufficiency leads to I(Na) deficit in vivo remains undefined. Mutations in pkp2 are detected in arrhythmogenic right ventricular cardiomyopathy (ARVC). Ventricular fibrillation and sudden death often occur in the 'concealed phase' of the disease, prior to overt structural damage. The mechanisms responsible for these arrhythmias remain poorly understood. We sought to characterize the morphology, histology, and ultrastructural features of PKP2-heterozygous-null (PKP2-Hz) murine hearts and explore the relation between PKP2 abundance, I(Na) function, and cardiac electrical synchrony.

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