Document Detail

The spectrum of cardiovascular anomalies in CHF1/Hey2 deficient mice reveals roles in endocardial cushion, myocardial and vascular maturation.
MedLine Citation:
PMID:  16242143     Owner:  NLM     Status:  MEDLINE    
CHF1/Hey2 null mice generated in different laboratories have discrepant cardiovascular phenotypes. To determine the effect of genetic background on phenotype, we backcrossed our knockout strain more than eight generations to the inbred strains BALB/c and C57BL/6. Knockout mice on these backgrounds showed disparate phenotypes. Mice on both backgrounds demonstrated ventricular septal defects (VSDs), tricuspid stenosis and mitral valve thickening, but at varying frequencies, suggesting a general defect in endocardial cushion remodeling. Additional defects seen exclusively on the C57BL/6 background included biventricular wall thinning and left ventricular enlargement, implying a more severe myocardial defect than previously observed. In addition, aortas and pulmonary arteries from these null mice had thinner walls. Intercrossing of the CHF1/Hey2 null mice on a C57BL/6 background with a C57BL/6 MLC2v-CHF1/Hey2 transgenic line overexpressing CHF1/Hey2 in the atrial and ventricular myocardium also rescued the VSD and myocardial phenotypes, but did not affect vascular wall thickness. Our results indicate that CHF1/Hey2 provides an important myocardial signal to the endocardial cushion for proper septation and valve formation and also plays an important role in maturation of the myocardium and vasculature.
Yasuhiko Sakata; Nobutaka Koibuchi; Fan Xiang; Joey M Youngblood; Caramai N Kamei; Michael T Chin
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2005-10-19
Journal Detail:
Title:  Journal of molecular and cellular cardiology     Volume:  40     ISSN:  0022-2828     ISO Abbreviation:  J. Mol. Cell. Cardiol.     Publication Date:  2006 Feb 
Date Detail:
Created Date:  2006-01-31     Completed Date:  2008-02-01     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0262322     Medline TA:  J Mol Cell Cardiol     Country:  England    
Other Details:
Languages:  eng     Pagination:  267-73     Citation Subset:  IM    
Vascular Medicine Research, Brigham and Women's Hospital and Harvard Medical School, 65 Landsdowne Street, Room 277, Cambridge, MA 02139, USA.
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MeSH Terms
Aorta / abnormalities
Basic Helix-Loop-Helix Transcription Factors / deficiency*,  genetics
Cardiovascular Abnormalities / genetics*
Endocardial Cushion Defects / genetics
Epigenesis, Genetic*
Gene Deletion
Heart / growth & development
Heart Ventricles / abnormalities
Mice, Inbred BALB C
Mice, Inbred C57BL
Mice, Knockout
Mitral Valve / abnormalities
Myocardium / metabolism
Repressor Proteins / genetics
Tricuspid Valve Stenosis / genetics
Tunica Media / abnormalities
Grant Support
R01 HL 67141-04/HL/NHLBI NIH HHS
Reg. No./Substance:
0/Basic Helix-Loop-Helix Transcription Factors; 0/Hey2 protein, mouse; 0/Repressor Proteins

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