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Development 2014 Aug 01;14115:3040-9. doi: 10.1242/dev.106518.
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Congenital heart disease protein 5 associates with CASZ1 to maintain myocardial tissue integrity.

Sojka S , Amin NM , Gibbs D , Christine KS , Charpentier MS , Conlon FL .

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 undergo cell movements associated with cardiac formation. We further report that CHD5 is essential for CASZ1 function and that the CHD5-CASZ1 interaction is necessary for cardiac morphogenesis. Collectively, these results establish a role for CHD5 and CASZ1 in the early stages of vertebrate cardiac development.

PubMed ID: 24993940
PMC ID: PMC4197678
Article link: Development
Grant support: [+]

Species referenced: Xenopus laevis
Genes referenced: actl6a casz1 chd5 eef1a2

Article Images: [+] show captions
References [+] :
Abbag, Congenital heart diseases and other major anomalies in patients with Down syndrome. 2006, Pubmed