Left-right asymmetry and cardiac looping: Implications for cardiac development and congenital heart disease

Citation
Is. Kathiriya et D. Srivastava, Left-right asymmetry and cardiac looping: Implications for cardiac development and congenital heart disease, AM J MED G, 97(4), 2000, pp. 271-279
Citations number
70
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Molecular Biology & Genetics
Journal title
AMERICAN JOURNAL OF MEDICAL GENETICS
ISSN journal
0148-7299 → ACNP
Volume
97
Issue
4
Year of publication
2000
Pages
271 - 279
Database
ISI
SICI code
0148-7299(200024)97:4<271:LAACLI>2.0.ZU;2-L
Abstract
Proper morphogenesis and positioning of internal organs requires delivery a nd interpretation of precise signals along the anterior-posterior, dorsal-v entral, and left-right axes. An elegant signaling cascade determines left- versus right-sided identity in visceral organs in a concordant fashion, res ulting in a predictable left-right (LR) organ asymmetry in all vertebrates. The complex morphogenesis of the heart and its connections to the vasculat ure are particularly dependent upon coordinated LR signaling pathways. Diso rganization of LR signals can result in myriad congenital heart defects tha t are a consequence of abnormal looping and remodeling of the primitive hea rt tube into a multi-chambered organ. A framework for understanding how LR asymmetric signals contribute to normal organogenesis has emerged and begin s to explain the basis of many human diseases of LR asymmetry. Here we revi ew the impact of LR signaling pathways on cardiac development and congenita l heart disease. Am. J. Med. Genet. (Semin. Med. Genet.) 97:271-279, 2000. (C) 2001 Wiley-Liss, Inc.