作者: JOSEF GEHRMANN , CHARLES I. BERUL
DOI: 10.1111/J.1540-8167.2000.TB01806.X
关键词: Connexin 、 Genetics 、 Biology 、 Transgene 、 Phenotype 、 Functional genomics 、 Cardiac conduction 、 Computational biology 、 Muscular dystrophy 、 Gene mutation 、 Gene targeting
摘要: The mouse has become the principal animal model for studying biologic processes in mammals. Major advances transgene and gene targeting technology enabled manipulation of genome a predictable fashion. Mutant strains provide important insights into molecular mechanisms underlying normal disordered cardiac conduction sudden death. A variety harboring mutations leading to inherited developmental disorders have been designed. Structural protein abnormalities, connexin defects, ion channelopathies associated with human clinical phenotypes, including congenital heart disease, cardiomyopathies, long QT syndrome, muscular dystrophy, engineered genome, creating models electrophysiologic disease. Functional analyses resultant phenotypes require appropriate sophisticated experimental methodology. In this review, genetic pertinent arrhythmogenic their application present-day ex vivo murine at whole organ levels are discussed.