Document Detail


Insights into sick sinus syndrome from an inducible mouse model.
MedLine Citation:
PMID:  21193513     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
AIMS: Sick sinus syndrome is a generalized abnormality of cardiac impulse formation and is responsible for a large proportion of pacemaker implantations. Although the exact aetiology is not known, it is widely accepted that age-dependent degenerative fibrosis of nodal tissue is the most common cause. Despite its importance, an animal model for sick sinus syndrome is lacking. We attempted to generate a mouse model phenocopying the pathohistological changes as well as the characteristic arrhythmic manifestations of this syndrome.
METHODS AND RESULTS: We crossed two genetically engineered mouse lines, ROSA-eGFP-DTA and HCN4-KiT-Cre, to achieve an inducible deletion of cells specifically in the cardiac pacemaking and conduction system. This deletion resulted in a degenerative fibrosis of nodal tissue, which accurately reflects the pathohistological findings in human sick sinus syndrome. The extent of the sino-atrial fibrosis could be controlled by varying the dosage of the inducing substance, tamoxifen. A high-dose protocol resulted in the complete ablation of all sino-atrial cells as demonstrated by histochemical analysis and quantitative reverse transcriptase-polymerase chain reaction. The animals developed a variety of arrhythmias, including progressive bradycardia, sinus pauses, supraventricular and ventricular tachycardia and chronotropic incompetence. Remarkably, the complete destruction of the primary pacemaker centre resulted in only a small increase in mortality.
CONCLUSION: This study describes the generation and analysis of an inducible mouse model which closely reflects the pathophysiological characteristics of sick sinus syndrome. The model, with the ability to control the extent of nodal cell ablation and fibrosis, offers new insights into sick sinus syndrome and other cardiac conduction diseases.
Authors:
Stefan Herrmann; Larissa Fabritz; Beate Layh; Paulus Kirchhof; Andreas Ludwig
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-12-30
Journal Detail:
Title:  Cardiovascular research     Volume:  90     ISSN:  1755-3245     ISO Abbreviation:  Cardiovasc. Res.     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-03-17     Completed Date:  2011-07-11     Revised Date:  2011-10-21    
Medline Journal Info:
Nlm Unique ID:  0077427     Medline TA:  Cardiovasc Res     Country:  England    
Other Details:
Languages:  eng     Pagination:  38-48     Citation Subset:  IM    
Affiliation:
Institut für Experimentelle und Klinische Pharmakologie und Toxikologie, Friedrich-Alexander-Universität Erlangen-Nürnberg, D-91054 Erlangen, Germany. herrmann@pharmakologie.uni-erlangen.de
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MeSH Terms
Descriptor/Qualifier:
Animals
Arrhythmias, Cardiac / genetics,  pathology,  physiopathology*
Biological Clocks*
Cyclic Nucleotide-Gated Cation Channels / genetics
Diphtheria Toxin / genetics
Disease Models, Animal
Electrocardiography
Fibrosis
Genes, Reporter
Green Fluorescent Proteins / genetics
Heart Rate*
Integrases / genetics
Mice
Mice, Transgenic
Peptide Fragments / genetics
Proteins / genetics
Proto-Oncogene Proteins c-kit / genetics
Sick Sinus Syndrome / genetics,  pathology,  physiopathology*
Sinoatrial Node / pathology,  physiopathology*
Telemetry
Time Factors
Chemical
Reg. No./Substance:
0/Cyclic Nucleotide-Gated Cation Channels; 0/Diphtheria Toxin; 0/Gt(ROSA)26Sor protein, mouse; 0/Peptide Fragments; 0/Proteins; 0/diphtheria toxin fragment A; 0/enhanced green fluorescent protein; 147336-22-9/Green Fluorescent Proteins; EC 2.7.10.1/Proto-Oncogene Proteins c-kit; EC 2.7.7.-/Cre recombinase; EC 2.7.7.-/Integrases
Comments/Corrections
Comment In:
Cardiovasc Res. 2011 Oct 1;92(1):178; author reply 179   [PMID:  21791463 ]
Cardiovasc Res. 2011 Apr 1;90(1):3-4   [PMID:  21335411 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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