Congenital heart disease protein 5 associates with CASZ1 to maintain myocardial tissue integrity.

ABSTRACT: The identification and characterization of the cellular and molecular pathways involved in the differentiation and morphogenesis of specific cell types of the developing heart are crucial to understanding the process of cardiac development and the pathology associated with human congenital heart disease. Here, we show that the cardiac transcription factor CASTOR (CASZ1) directly interacts with congenital heart disease 5 protein (CHD5), which is also known as tryptophan-rich basic protein (WRB), a gene located on chromosome 21 in the proposed region responsible for congenital heart disease in individuals with Down's syndrome. We demonstrate that loss of CHD5 in Xenopus leads to compromised myocardial integrity, improper deposition of basement membrane, and a resultant failure of hearts to u

SUBMITTER: Sojka S 

PROVIDER: S-EPMC4197678 | biostudies-literature | 2014 Aug

REPOSITORIES: biostudies-literature