Accession Number:



Cardiomyocyte Chirality Defects in Congenital Heart Disease

Descriptive Note:

Technical Report,01 May 2018,30 Apr 2019

Corporate Author:

The Trustees of Columbia University New York United States

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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 utilized our genetic and patient based stem cells lines to understand the cellular differences that occur in heterotaxy. Specifically we characterize fundamental differences in cell architecture and adhesion and the signaling cascades that underpin them.

Subject Categories:

  • Medicine and Medical Research

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