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Titolo:
Ionic mechanisms responsible for the electrocardiographic phenotype of theBrugada syndrome are temperature dependent
Autore:
Dumaine, R; Towbin, JA; Brugada, P; Vatta, M; Nesterenko, DV; Nesterenko, VV; Brugada, J; Brugada, R; Antzelevitch, C;
Indirizzi:
Masonic Med Res Lab, Dept Mol Biol, Utica, NY 13501 USA Masonic Med Res Lab Utica NY USA 13501 Dept Mol Biol, Utica, NY 13501 USA Masonic Med Res Lab, Dept Expt Cardiol, Utica, NY 13501 USA Masonic Med Res Lab Utica NY USA 13501 Expt Cardiol, Utica, NY 13501 USA Baylor Coll Med, Dept Pediat, Houston, TX 77030 USA Baylor Coll Med Houston TX USA 77030 , Dept Pediat, Houston, TX 77030 USA Baylor Coll Med, Dept Med, Houston, TX 77030 USA Baylor Coll Med Houston TX USA 77030 Med, Dept Med, Houston, TX 77030 USA Olv Hosp, Ctr Cardiovasc, Aalst, Belgium Olv Hosp Aalst BelgiumOlv Hosp, Ctr Cardiovasc, Aalst, Belgium Univ Barcelona, Cardiovasc Inst, Hosp Clin, Barcelona, Spain Univ Barcelona Barcelona Spain iovasc Inst, Hosp Clin, Barcelona, Spain
Titolo Testata:
CIRCULATION RESEARCH
fascicolo: 9, volume: 85, anno: 1999,
pagine: 803 - 809
SICI:
0009-7330(19991029)85:9<803:IMRFTE>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
CANINE VENTRICULAR EPICARDIUM; INHERITED CARDIAC-ARRHYTHMIA; TRANSIENT OUTWARD CURRENT; LONG-QT SYNDROME; MOLECULAR MECHANISM; SODIUM-CHANNELS; MYOCYTES; INACTIVATION; MUTATIONS; DISEASE;
Keywords:
Brugada syndrome; Na+ channel; temperature;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
30
Recensione:
Indirizzi per estratti:
Indirizzo: Dumaine, R Masonic Med Res Lab, Dept Mol Biol, 2150 Bleecker St, Utica, NY13501 USA Masonic Med Res Lab 2150 Bleecker St Utica NY USA 13501 501 USA
Citazione:
R. Dumaine et al., "Ionic mechanisms responsible for the electrocardiographic phenotype of theBrugada syndrome are temperature dependent", CIRCUL RES, 85(9), 1999, pp. 803-809

Abstract

The Brugada syndrome is a major cause of sudden death, particularly among young men of Southeast Asian and Japanese origin. The syndrome is characterized electrocardiographically by an ST-segment elevation in V1 through V3 and a rapid polymorphic ventricular tachycardia that can degenerate into ventricular fibrillation. Our group recently linked the disease to mutations in SCN5A, the gene encoding for the cu subunit of the cardiac sodium channel. When heterologously expressed in frog oocytes, electrophysiological data recorded from the Thr1620Met missense mutant failed to adequately explain the electrocardiographic phenotype. Therefore, we sought to further characterize the electrophysiology of this mutant. We hypothesized that at more physiological temperatures, the missense mutation may change the gating of thesodium channel such that the net outward current is dramatically augmentedduring the early phases of the right ventricular action potential. In the present study, we test this hypothesis by expressing Thr1620Met in a mammalian cell line, using the patch-clamp technique to study the currents at 32 degrees C. Our results indicate that Thr1620Met current decay kinetics are faster when compared with the wild type at 32 degrees C. Recovery from inactivation was slower for Thr1620Met at 32 degrees C, and steady-state activation was significantly shifted. Our findings explain the features of the ECG of Brugada patients, illustrate for the first time a cardiac sodium channel mutation of which the arrhythmogenicity is revealed only at temperaturesapproaching the physiological range, and suggest that some patients may bemore at risk during febrile states.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 31/03/20 alle ore 16:29:55