Generation & Characterization
of a Mouse Model for a Sodium Channel
Based Long QT Syndrome (LQT3),
an Inherited Arrhythmogenic Disease

By Dieter Nuyens
Acta Biomedica Lovaniensia, No. 357
June 2006
Leuven University Press
ISBN: 9058675300
124 pages, Illustrated, 6 ¼” x 9 ½”
$79.50 Paper Original

Contents include:
Table of contents
Figures and tables
Chapter 1: Introduction
1.1. General overview
1.2. Some history
1.3. Long QT, what's in a name?
1.4. LQTS, a matter of genes
1.5. Genotype-phenotype correlations
1.6. The Sudden Infant Death Syndrome: the role of LQT3
1.7. Current therapeutic possibilities and limitations
1.8. The cardiac sodium channel and LQT3
1.9. General pathophysiological mechanism of LQT3
Chapter 2: Aims
Chapter 3: Methods
3.1. Construction of a 'knock-in' targeting vector
3.2. Homologous and site-specific Cre/loxP-mediated recombination in embryonic stem cells
3.3. Generation of chimeric and germline mice
3.4. Genotyping procedures and expression analysis
3.5. Developmental and histological analysis
3.6. Animal experiments
3.7. Action potential measurements
3.8. Sodium current measurements
3.9. Statistical analysis
Chapter 4: Results
4.1. Generation of SCN5A mice
4.2. Prolonged repolarization in SCN5A mice
4.3. Increased arrhythmogenicity in SCN5A mice
4.4. Adrenergic agonists are anti-arrhythmic in SCN5A mice
4.5. Other electrocardiographic abnormalities in SCN5A mice
Chapter 5: Discussion
5.1. SCN5A mice constitute a LQT3 model
5.2. LQT3 arrhythmias in mice and man
5.3. Novel characteristics of the LQT3 syndrome
5.4. Mechanistic insights in LQT3 induced arrhythmias
5.5. Medical implications
Curriculum Vitae


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