Document Detail

Pitx2c patterns anterior myocardium and aortic arch vessels and is required for local cell movement into atrioventricular cushions.
MedLine Citation:
PMID:  12397115     Owner:  NLM     Status:  MEDLINE    
Inactivation of the left-right asymmetry gene Pitx2 has been shown, in mice, to result in right isomerism with associated defects that are similar to that found in humans. We show that the Pitx2c isoform is expressed asymmetrically in a presumptive secondary heart field within the branchial arch and splanchnic mesoderm that contributes to the aortic sac and conotruncal myocardium. Pitx2c was expressed in left aortic sac mesothelium and in left splanchnic and branchial arch mesoderm near the junction of the aortic sac and branchial arch arteries. Mice with an isoform-specific deletion of Pitx2c had defects in asymmetric remodeling of the aortic arch vessels. Fatemapping studies using a Pitx2 cre recombinase knock-in allele showed that daughters of Pitx2-expressing cells populated the right and left ventricles, atrioventricular cushions and valves and pulmonary veins. In Pitx2 mutant embryos, descendents of Pitx2-expressing cells failed to contribute to the atrioventricular cushions and valves and the pulmonary vein, resulting in abnormal morphogenesis of these structures. Our data provide functional evidence that the presumptive secondary heart field, derived from branchial arch and splanchnic mesoderm, patterns the forming outflow tract and reveal a role for Pitx2c in aortic arch remodeling. Moreover, our findings suggest that a major function of the Pitx2-mediated left right asymmetry pathway is to pattern the aortic arches, outflow tract and atrioventricular valves and cushions.
Chengyu Liu; Wei Liu; Jennifer Palie; Mei Fang Lu; Nigel A Brown; James F Martin
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Development (Cambridge, England)     Volume:  129     ISSN:  0950-1991     ISO Abbreviation:  Development     Publication Date:  2002 Nov 
Date Detail:
Created Date:  2002-10-24     Completed Date:  2002-12-03     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  8701744     Medline TA:  Development     Country:  England    
Other Details:
Languages:  eng     Pagination:  5081-91     Citation Subset:  IM    
Alkek Institute of Biosciences and Technology, Texas A&M System Health Science Center, 2121 Holcombe Blvd, Houston 77030, USA.
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MeSH Terms
Aorta, Thoracic / embryology*
Atrioventricular Node / cytology,  embryology
Body Patterning / genetics,  physiology
Branchial Region / embryology
Cell Movement / genetics,  physiology
Gene Expression Regulation, Developmental
Genes, Homeobox
Heart / embryology*
Homeodomain Proteins / genetics,  physiology*
Mice, Inbred C57BL
Mice, Knockout
Mice, Transgenic
Neural Crest / cytology,  embryology
Pulmonary Veins / embryology
Xenopus Proteins*
Grant Support
Reg. No./Substance:
0/Homeodomain Proteins; 0/Pitx2c protein, Xenopus; 0/Xenopus Proteins

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

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