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Mapping of a Gene for Long QT Syndrome to Chromosome 4q25-27
Authors:Jean-Jacques Schott  Flavien Charpentier  Sophie Peltier  Patrick Foley  Emmanuel Drouin  Jean-Brieuc Bouhour  Patricia Donnelly  Gilles Vergnaud  Lucien Bachner  Jean-Paul Moisan  Herv Le Marec  and Olivier Pascal
Institution:Jean-Jacques Schott, Flavien Charpentier, Sophie Peltier, Patrick Foley, Emmanuel Drouin, Jean-Brieuc Bouhour, Patricia Donnelly, Gilles Vergnaud, Lucien Bachner, Jean-Paul Moisan, Hervé Le Marec, and Olivier Pascal
Abstract:Long QT syndrome (LQTS) is a heterogeneous inherited disorder causing syncope and sudden death from ventricular arrhythmias. A first locus for this disorder was mapped to chromosome 11p15.5. However, locus heterogeneity has been demonstrated in several families, and two other loci have recently been located on chromosomes 7q35-36 and 3p21-24. We used linkage analysis to map the locus in a 65-member family in which LQTS was associated with more marked sinus bradycardia than usual, leading to sinus node dysfunction. Linkage to chromosome 11p15.5, 7q35-36, or 3p21-24 was excluded. Positive linkage was obtained for markers located on chromosome 4q25-27. A maximal LOD score of 7.05 was found for marker D4S402. The identification of a fourth locus for LQTS confirms its genetic heterogeneity. Locus 4q25-27 is associated with a peculiar phenotype within the LQTS entity.
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