Research Topics
Species | M C SanguinettiSummaryAffiliation: University of Utah Country: USA Publications
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Detail Information
Publications
Coassembly of K(V)LQT1 and minK (IsK) proteins to form cardiac I(Ks) potassium channelM C Sanguinetti
Department of Medicine, Cardiology Division, University of Utah, Salt Lake City 84112, USA
Nature 384:80-3. 1996..Coexpression of K(V)LQT1 with minK induced a current that was almost identical to cardiac I(Ks). Therefore, K(V)LQT1 is the subunit that coassembles with minK to form I(Ks) channels and I(Ks) dysfunction is a cause of cardiac arrhythmia...- Potassium channelopathiesM C Sanguinetti
Department of Medicine, University of Utah, Salt Lake City 84112, USA
Neuropharmacology 36:755-62. 1997..An understanding of the molecular basis of these diseases could facilitate the discovery and development of specific pharmacological therapies... - The S4-S5 linker couples voltage sensing and activation of pacemaker channelsJ Chen
Department of Medicine, Division of Cardiology, Eccles Program in Human Molecular Biology and Genetics, University of Utah, Eccles Institute of Human Genetics, 15 N 2030 E, Room 4220, Salt Lake City, UT 84112, USA
Proc Natl Acad Sci U S A 98:11277-82. 2001..Our findings also suggest that opening of HCN and related channels corresponds to activation of a gate located near the inner pore, rather than recovery of channels from a C-type inactivated state... - Long-QT syndrome-associated missense mutations in the pore helix of the HERG potassium channelF D Huang
Department of Medicine, Division of Cardiology, University of Utah, Salt Lake City 84112, USA
Circulation 104:1071-5. 2001..CONCLUSIONS: These findings define the physiological consequences of mutations in HERG that cause LQTS and indicate the importance of the pore helix of HERG for normal channel function... - Mutations of the S4-S5 linker alter activation properties of HERG potassium channels expressed in Xenopus oocytesM C Sanguinetti
Eccles Program in Human Molecular Biology and Genetics, Division of Cardiology, Department of Medicine, University of Utah, Salt Lake City, UT 84112
J Physiol 514:667-75. 1999..5. The results indicate that the S4-S5 linker is a crucial component of the activation gate of HERG channels... - Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivationJ Chen
Department of Medicine, Division of Cardiology, University of Utah, Salt Lake City, Utah 84112, USA
J Biol Chem 274:10113-8. 1999..The location of these mutations suggests they may disrupt the PAS domain and interfere with its interaction with the S4-S5 linker of the HERG channel... - Trapping of a methanesulfonanilide by closure of the HERG potassium channel activation gateJ S Mitcheson
Department of Medicine, Division of Cardiology, Eccles Institute of Human Genetics, University of Utah, Salt Lake City, Utah 84112, USA
J Gen Physiol 115:229-40. 2000..The ability of HERG channels to trap MK-499, despite its large size, suggests that the vestibule of this channel is larger than the well studied Shaker K(+) channel... - A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channelM C Sanguinetti
Eccles Program in Human Molecular Biology and Genetics, University of Utah Health Sciences Center, Salt Lake City 84112, USA
Cell 81:299-307. 1995..Since block of IKr is a known mechanism for drug-induced cardiac arrhythmias, the finding that HERG encodes IKr channels provides a mechanistic link between certain forms of inherited and acquired LQT... - Long QT syndrome-associated mutations in the S4-S5 linker of KvLQT1 potassium channels modify gating and interaction with minK subunitsL Franqueza
Department of Medicine, Division of Cardiology, University of Utah, Salt Lake City, Utah 84112, USA
J Biol Chem 274:21063-70. 1999..The decrease in I(Ks) caused by loss of function or altered gating properties explains the prolonged QT interval and increased risk of arrhythmia and sudden death associated with these mutations in KVLQT1... - Functional roles of charged residues in the putative voltage sensor of the HCN2 pacemaker channelJ Chen
Department of Medicine, Division of Cardiology and Eccles Program in Human Molecular Biology and Genetics, University of Utah, Salt Lake City, Utah 84112 5330, USA
J Biol Chem 275:36465-71. 2000.... - Mutations in the hminK gene cause long QT syndrome and suppress IKs functionI Splawski
Department of Human Genetics, University of Utah, Salt Lake City 84112, USA
Nat Genet 17:338-40. 1997..This is the first description of KCNE1 as an LQT gene and confirms that hminK is an integral protein of the IKs channel... - Dysfunction of delayed rectifier potassium channels in an inherited cardiac arrhythmiaM C Sanguinetti
Department of Medicine, University of Utah, Salt Lake City 84112, USA
Ann N Y Acad Sci 868:406-13. 1999..The functional consequences of mutations in delayed rectifier K+ channel subunits are delayed cardiac repolarization, lengthened QT interval, and an increased risk of torsade de pointes and sudden death... - Molecular biology of K(+) channels and their role in cardiac arrhythmiasM Tristani-Firouzi
Department of Medicine, Division of Cardiology, University of Utah, Salt Lake City, Utah 84112, USA
Am J Med 110:50-9. 2001..However, many of these same drugs, as well as other common medications that are structurally unrelated, can also cause long QT syndrome and induce ventricular arrhythmia... - Long QT syndrome: ionic basis and arrhythmia mechanism in long QT syndrome type 1M C Sanguinetti
Department of Medicine, University of Utah, Salt Lake City 84112, USA
J Cardiovasc Electrophysiol 11:710-2. 2000..It remains to be proven that an understanding of the molecular basis of LQT1 will lead to more effective therapy...