Mouse Model of SCN5A -Linked Hereditary Lenègre’s Disease
نویسندگان
چکیده
منابع مشابه
Mouse model of SCN5A-linked hereditary Lenègre's disease: age-related conduction slowing and myocardial fibrosis.
BACKGROUND We have previously linked hereditary progressive cardiac conduction defect (hereditary Lenègre's disease) to a loss-of-function mutation in the gene encoding the main cardiac Na+ channel, SCN5A. In the present study, we investigated heterozygous Scn5a-knockout mice (Scn5a+/- mice) as a model for hereditary Lenègre's disease. METHODS AND RESULTS In Scn5a+/- mice, surface ECG recordi...
متن کاملHaploinsufficiency in combination with aging causes SCN5A-linked hereditary Lenègre disease.
OBJECTIVES The goal of this study was to investigate the genotype-to-phenotype relationship between SCN5A gene mutation and progressive cardiac conduction defect in order to gain insights into the pathophysiologic mechanisms of the disease. BACKGROUND Progressive cardiac conduction defect is a frequent disease commonly attributed to degeneration and fibrosis of the His bundle and its branches...
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ژورنال
عنوان ژورنال: Circulation
سال: 2005
ISSN: 0009-7322,1524-4539
DOI: 10.1161/01.cir.0000160853.19867.61