Key result
Rbm24 deficiency resulted in a reduction in sarcomeric proteins, Z-disc abnormality, diminished heart contractility, and absence of circulation in zebrafish embryos.
The RNA-binding protein Rbm24 plays a crucial role in regulating cardiac gene expression, sarcomeric assembly, and cardiac contractility, suggesting a potential novel pathway to cardiomyopathy.
RBM24 loss-of-function findings in animals are hypothesis-generating for cardiomyopathy; leaves open human relevance and therapeutic potential.
AIMS: The factors responsible for cardiomyopathy are not fully understood. Our studies of the transcriptome of human embryonic stem cell-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 in sarcomeric proteins, Z-disc abnormality, and diminished heart contractility, resulting in the 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. CONCLUSION: We identified a novel role of the tissue-specific RNA-binding protein (RBP) Rbm24 involving in the regulation of cardiac gene expression, sarcomeric assembly, and cardiac contractility. This study uncovers a potential novel pathway to cardiomyopathy through down-regulation of the RBP Rbm24.
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Poon et al. (2012) studied Cardiomyopathy and heart development. Rbm24 deficiency vs. Normal Rbm24 expression was evaluated on Sarcomere assembly, heart contractility, and circulation. Rbm24 deficiency resulted in a reduction in sarcomeric proteins, Z-disc abnormality, diminished heart contractility, and absence of circulation in zebrafish embryos.
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