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Handbook of Experimental Pharmacology - Part 9

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Hiện IKs được tăng cường bởi sự kích thích β-adrenergic (Walsh và Kass 1988), α-adrenergic kích thích, phosphoryl hóa PKC, hoặc tăng trong [Ca2 +] i (Tohse et al. 1987) Kích hoạt các thụ thể β-adrenergic tăng pKa hoạt động | HEP 2006 171 287-304 Springer-Verlag Berlin Heidelberg 2006 Mutation-Specific Pharmacology of the Long QT Syndrome R.S. Kass1 E A.J. Moss2 Department of Pharmacology Columbia University College of Physicians and Surgeons New York NY 10032 USA RSK20@Columbia.edu 2Heart Research Follow-up Program Department of Medicine University of Rochester School of Medicine and Dentistry Rochester NY 14642 USA 1 Background. 288 2 Arrhythmia Risk Factors Are Mutation Gene-Specific. 289 3 Mutation-Specific Pharmacology Role of the Sodium Channel. 290 4 Na Channel Block by Local Anesthetics Is Linked to Channel Inactivation . 291 5 LQT-3 Mutations A Common Phenotype Caused by a Range of Mutation-Induced Channel Function. 292 6 Clinical Relevance of Mutations Within Different Regions of the Ion Channel Structure Function. 294 7 Basic Electrophysiology Revealed Through LQTS Studies. 296 8 Identification of Cardiac Delayed Rectifier Channels. 296 9 The Cardiac Sodium Channel and the Action Potential Plateau Phase. 298 10 The Sodium Channel Inactivation Gate as a Molecular Complex. 298 11 Summary and Future Directions. 299 References . 300 Abstract The congenital long QT syndrome is a rare disease in which inherited mutations of genes coding for ion channel subunits or channel interacting proteins delay repolarization of the human ventricle and predispose mutation carriers to the risk of serious or fatal arrhythmias. Though a rare disorder the long QT syndrome has provided invaluable insight from studies that have bridged clinical and pre-clinical basic science medicine. In this brief review we summarize some of the key clinical and genetic characteristics of this disease and highlight novel findings about ion channel structure function and the causal relationship between channel dysfunction and human disease that have come from investigations of this disorder. Mutation-Specific Pharmacology of the Long QT Syndrome 293 cations for triggers of this unique mutation Tateyama et al. 2003 .