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rdf:type |
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lifeskim:mentions |
umls-concept:C0085298,
umls-concept:C0205314,
umls-concept:C0243067,
umls-concept:C0282637,
umls-concept:C0314603,
umls-concept:C0457405,
umls-concept:C0679622,
umls-concept:C1522565,
umls-concept:C1704259,
umls-concept:C1705165,
umls-concept:C1705987,
umls-concept:C2700061
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pubmed:issue |
5
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pubmed:dateCreated |
2000-10-4
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pubmed:abstractText |
HF-1 b, an SP1 -related transcription factor, is preferentially expressed in the cardiac conduction system and ventricular myocytes in the heart. Mice deficient for HF-1 b survive to term and exhibit normal cardiac structure and function but display sudden cardiac death and a complete penetrance of conduction system defects, including spontaneous ventricular tachycardia and a high incidence of AV block. Continuous electrocardiographic recordings clearly documented cardiac arrhythmogenesis as the cause of death. Single-cell analysis revealed an anatomic substrate for arrhythmogenesis, including a decrease and mislocalization of connexins and a marked increase in action potential heterogeneity. Two independent markers reveal defects in the formation of ventricular Purkinje fibers. These studies identify a novel genetic pathway for sudden cardiac death via defects in the transition between ventricular and conduction system cell lineages.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Connexins,
http://linkedlifedata.com/resource/pubmed/chemical/DNA-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Potassium,
http://linkedlifedata.com/resource/pubmed/chemical/Potassium Channels,
http://linkedlifedata.com/resource/pubmed/chemical/Potassium Channels, Voltage-Gated,
http://linkedlifedata.com/resource/pubmed/chemical/RNA, Messenger,
http://linkedlifedata.com/resource/pubmed/chemical/Sp4 Transcription Factor,
http://linkedlifedata.com/resource/pubmed/chemical/Sp4 protein, rat,
http://linkedlifedata.com/resource/pubmed/chemical/connexin 40,
http://linkedlifedata.com/resource/pubmed/chemical/potassium channel protein I(sk)
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pubmed:status |
MEDLINE
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pubmed:month |
Sep
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pubmed:issn |
0092-8674
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pubmed:author |
pubmed-author:Barrere-LemaireSS,
pubmed-author:BrownA BAB,
pubmed-author:ChienK RKR,
pubmed-author:ClarkR BRB,
pubmed-author:FellG SGS,
pubmed-author:FiserAA,
pubmed-author:GilesW RWR,
pubmed-author:GourdieR GRG,
pubmed-author:KondoRR,
pubmed-author:KubalakS WSW,
pubmed-author:MinamisawaSS,
pubmed-author:Nguyên-TrânV TVT,
pubmed-author:NormanL WLW,
pubmed-author:RahmeM MMM,
pubmed-author:Ruiz-LozanoPP,
pubmed-author:WollertK CKC
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pubmed:issnType |
Print
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pubmed:day |
1
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pubmed:volume |
102
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
671-82
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:11007485-Action Potentials,
pubmed-meshheading:11007485-Alleles,
pubmed-meshheading:11007485-Animals,
pubmed-meshheading:11007485-Cell Count,
pubmed-meshheading:11007485-Cell Lineage,
pubmed-meshheading:11007485-Connexins,
pubmed-meshheading:11007485-DNA-Binding Proteins,
pubmed-meshheading:11007485-Death, Sudden, Cardiac,
pubmed-meshheading:11007485-Electric Conductivity,
pubmed-meshheading:11007485-Electrocardiography,
pubmed-meshheading:11007485-Female,
pubmed-meshheading:11007485-Gene Deletion,
pubmed-meshheading:11007485-Heart Block,
pubmed-meshheading:11007485-Heart Conduction System,
pubmed-meshheading:11007485-Heart Ventricles,
pubmed-meshheading:11007485-Male,
pubmed-meshheading:11007485-Mice,
pubmed-meshheading:11007485-Mice, Knockout,
pubmed-meshheading:11007485-Penetrance,
pubmed-meshheading:11007485-Potassium,
pubmed-meshheading:11007485-Potassium Channels,
pubmed-meshheading:11007485-Potassium Channels, Voltage-Gated,
pubmed-meshheading:11007485-Purkinje Fibers,
pubmed-meshheading:11007485-RNA, Messenger,
pubmed-meshheading:11007485-Radio,
pubmed-meshheading:11007485-Sp4 Transcription Factor,
pubmed-meshheading:11007485-Tachycardia, Ventricular,
pubmed-meshheading:11007485-Telemetry
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pubmed:year |
2000
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pubmed:articleTitle |
A novel genetic pathway for sudden cardiac death via defects in the transition between ventricular and conduction system cell lineages.
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pubmed:affiliation |
UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego, La Jolla 92093, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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