Dec 5, 2000

Transcriptional regulation of cardiac development: implications for congenital heart disease and DiGeorge syndrome

Pediatric Research
Jonathan A Epstein, C A Buck

Abstract

In recent years, impressive advances have occurred in our understanding of transcriptional regulation of cardiac development. These insights have begun to elucidate the mystery of congenital heart disease at the molecular level. In addition, the molecular pathways emerging from the study of cardiac development are being applied to the understanding of adult cardiac disease. Preliminary results support the contention that a thorough understanding of molecular programs governing cardiac morphogenesis will provide important insights into the pathogenesis of human cardiac diseases. This review will focus on examples of transcription factors that play critical roles at various phases of cardiac development and their relevance to cardiac disease. This is an exciting and burgeoning area of investigation. It is not possible to be all-inclusive, and the reader will note important efforts in the areas of cardiomyocyte determination, left-right asymmetry, cardiac muscular dystrophies, electrophysiology and vascular disease are not covered. For a more complete discussion, the reader is referred to recent reviews including the excellent compilation of observations assembled by Harvey and Rosenthal (1).

Mentioned in this Paper

Cell Motility
Porcine Reproductive and Respiratory Syndrome
Transcription, Genetic
Shprintzen Syndrome
Gestational Age
Multiple Congenital Anomalies
Congenital Heart Defects
Heart Valves
Cell Lineage
Deletion Mutation

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