000070799 001__ 70799
000070799 005__ 20180530112558.0
000070799 037__ $$aCOMPON-2018-0005
000070799 041__ $$aeng
000070799 100__ $$00000-0002-1927-1762$$aSampedro-Puente, Adolfo$$u
000070799 245__ $$aDifferential Responses to Beta-Adrenergic Stimulation in the Long-QT Syndrome Type 1: Characterization and Mechanisms
000070799 260__ $$c2017
000070799 506__ $$aAccess copy available to the general public$$fUnrestricted
000070799 5203_ $$aLong QT syndrome type 1 (LQT1) is caused by muta- tions that impair the function of the slow delayed rectifier potassium (IK s ) channels. Most LQT1 patients experience arrhythmic events during beta-adrenergic stimulation (β- AS). A full description of the ionic mechanisms underlying arrhythmogenecity in LQT1 patients and their relation to β-AS is still lacking. In this study we constructed a set of stochastic human ventricular cell models reproducing ex- perimental AP properties at baseline and following ionic inhibitions. Using the constructed models, we showed that AP duration, morphology and beat-to-beat variability in LQT1 are highly specific of the underlying ionic character- istics. Likewise, the response of individual IK s -deficient cells to β-AS can range from negligible to as much as 200% increase in AP temporal variability, recognized as a marker of arrhythmogenesis in the setting of LQT1. By partial correlation analysis, major ionic factors driving AP changes associated with LQT1 and β-AS were ascer- tained.
000070799 536__ $$9info:eu-repo/grant/Agreement/ES/MINECO TIN2013-41998-R$$9info:eu-repo/grant/Agreement/ES/MINECO DPI2016-75458-R$$9info:eu-repo/grant/Agreement/EU/ERC-2014-StG-638284
000070799 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/
000070799 6531_ $$aLong-QT
000070799 6531_ $$aBeta-Adrenergic Stimulation  
000070799 655_4 $$aComunicación
000070799 700__ $$0
0000-0001-5175-3166$$aFernández-Bes, Jesús$$u
000070799 700__ $$0
0000-0002-1960-407X$$aPueyo, Esther$$u
000070799 773__ $$dRennes$$gComputing in Cardiology (2017, Rennes, France)
000070799 8564_ $$s429985$$uhttp://zaguan.unizar.es/record/70799/files/COMPON-2018-0005.pdf
000070799 909CO $$ooai:zaguan.unizar.es:70799$$pcomunicaciones-ponencias
000070799 980__ $$aCOMPON