Prox1 maintains muscle structure and growth in the developing heart

• DOI: 10.1242/dev.030007
• Publication Date: 2008-12-18T01:25:04Z
• Authors: Catherine A. Risebro; Richelle G. Searles; Athalie A. D. Mel...; Elisabeth Ehler; Nipurna Jina; Sonia Shah; Jacky Pallas; Mike Hubank; Miriam Dillard; Natasha L. Harvey; Robert J. Schwartz; Kenneth R. Chien; Guillermo Oliver; Paul R. Riley
• Keywords: Myofibril; Heart development

Impaired cardiac muscle growth and aberrant myocyte arrangement underlie congenital heart disease cardiomyopathy. We show that cardiac-specific inactivation of the murine homeobox transcription factor Prox1 results in disruption expression localisation sarcomeric proteins, gross myofibril disarray growth-retarded hearts. Furthermore, we demonstrate is required for direct transcriptional regulation genes encoding structural proteins α-actinin, N-RAP zyxin, which collectively function to maintain an actin-α-actinin interaction as fundamental association sarcomere. Aspects abnormal development manifestation a subset muscular-based have previously been attributed mutations key proteins. Our study reveals essential requirement sarcomere integrity, context enabling foetal cardiomyocyte hypertrophy, maintenance contractile progression towards inherited or acquired myopathic disease.