Cardiomyocyte Chirality Defects In Congenital Heart Disease
Abstract
A large proportion of congenital heart disease is caused by a defect in correct partitioning of the left and right compartments of the cardiac mesoderm. The result of this failure of laterality is a wide assortment of abnormal atrial, ventricular and arterial relationships. Aberration of early left right patterning is the underlying cause of heterotaxy. The focus of this grant is to use induced pluripotent stem cells to model and understand inherent cellular laterality. We have generated a genetic model of heterotaxy using a CRISPR interference system that targets the expression of a transcription factor, ZIC3, that has been implicated in inherited versions of heterotaxy. During this reporting period, we have also derived patient stem cell lines from patients with heterotaxy and no known genetic mutations. From these cell lines we characterized defects in cytoskeletal organization and gene expression differences that may underpin the development of heterotaxy.
Document Details
- Document Type
- Technical Report
- Publication Date
- May 01, 2018
- Accession Number
- AD1055804
Entities
People
- Fine Barry