ZFIN ID: ZDB-PUB-120223-14
RNA-binding protein RBM24 is required for sarcomere assembly and heart contractility
Poon, K.L., Tan, K.T., Wei, Y.Y., Ng, C.P., Colman, A., Korzh, V., and Xu, X.Q.
Date: 2012
Source: Cardiovascular research   94(3): 418-427 (Journal)
Registered Authors: Korzh, Vladimir, Poon, Kar Lai
Keywords: none
MeSH Terms:
  • Animals
  • Cell Differentiation
  • Embryonic Stem Cells/cytology
  • Embryonic Stem Cells/metabolism
  • Gene Expression Profiling
  • Gene Expression Regulation, Developmental*
  • Gene Knockdown Techniques
  • Heart/embryology*
  • Humans
  • Myocardial Contraction*
  • Myocytes, Cardiac/metabolism*
  • RNA-Binding Proteins/genetics
  • RNA-Binding Proteins/metabolism*
  • Sarcomeres/metabolism*
  • Zebrafish/anatomy & histology
  • Zebrafish/embryology*
  • Zebrafish/genetics
  • Zebrafish/metabolism
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/metabolism*
PubMed: 22345307 Full text @ Cardiovasc. Res.

Aims The factors responsible for cardiomyopathy are not fully understood. Our studies of the transcriptome of human embryonic stem cells (hESC)-derived cardiomyocytes identified novel genes up-regulated during cardiac differentiation including RBM24 . We therefore studied how its deficiency affected heart development.

Methods and Results The expression of Rbm24 was detected in mouse cardiomyocytes and embryonic myocardium of zebrafish at the RNA and protein level. The Rbm24 loss-of-function showed that Rbm24 deficiency resulted in a reduction of sarcomeric proteins, Z discs abnormality and diminished heart contractility resulting in absence of circulation in zebrafish embryos. Gene expression profiling revealed down-regulation of multiple pathways associated with sarcomere assembly and vasculature development in Rbm24 deficiency.

Conclusions We identified a novel role of the tissue specific RNA binding protein Rbm24 involving regulation of cardiac gene expression, sarcomeric assembly and cardiac contractility. This study uncovers a potential novel pathway to cardiomyopathy through down-regulation of the RNA-binding protein Rbm24.