Developmental and genetic aspects of congenital heart... : Current Opinion in Cardiology (original) (raw)
Molecular Genetics
Department of Pediatrics, Division of Cardiology, and Department of Molecular Biology and Oncology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
Correspondence to D. Srivastava, MD, Department of Pediatrics, Division of Cardiology, and Department of Molecular Biology and Oncology, University of Texas Southwestern Medical Center, 6000 Harry Hines Boulevard, Room NA 8.124, Dallas, TX 75235–9148, USA; e-mail: [email protected]
Abstract
Congenital heart defects (CHDs) are the result of abnormal cardiac mesoderm or cardiac neural crest development. The molecular cause of most congenital heart disease remains unknown, although numerous cardiac regulatory factors have recently been described. dHAND and eHAND are basic helix-loop-helix transcription factors expressed differentially in the right and left ventricles, respectively, and in the cardiac neural crest. Mice lacking dHAND have a hypoplastic right ventricle and abnormal development of vessels arising from the heart and cell death of craniofacial precursors. By searching for dHAND-dependent genes, a gene likely responsible for the cardiac and craniofacial defects associated with chromosome 22q11 deletion has been identified. A systematic dissection of molecular pathways involved in cardiogenesis should allow for further identification of genes responsible for CHD.
© 1999 Lippincott Williams & Wilkins, Inc.