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Exploring the Activities of RBPMS Proteins in Myocardial Biology

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Abstract

Numerous RNA-binding proteins (RBPs) are expressed in the heart, and mutations in several RBPs have been implicated in cardiovascular disease through genetic associations, animal modeling, and mechanistic studies. However, the functions of many more cardiac RBPs, and their relevance to disease states, remain to be elucidated. Recently, we have initiated studies to characterize the functions of the RBPs RBPMS and RBPMS2 in regulating myocardial biology in zebrafish and higher vertebrate species. These studies began when we learned, using an unbiased gene discovery approach, that rbpms2a and rbpms2b in zebrafish are robust markers of embryonic myocardium. This observation, which is consistent with published data, suggests that the encoded proteins are likely to be performing critical functions in regulating one or more aspects of cardiomyocyte differentiation, proliferation, survival, and/or contractility. This notion is supported by recent reports demonstrating that zebrafish embryos with disrupted Rbpms2 function exhibit gross signs of cardiac distress. Interestingly, a 20-year-old study determined that myocardial tissue from the frog, chick, and mouse also express high levels of Rbpms and/or Rbpms2, which is suggestive of evolutionary conservation of function. In this review, we will provide a historical account of how RBPMS and RBPMS2 genes were discovered, attempt to clarify some potentially confusing nomenclature, and summarize published observations that inform our ongoing studies.

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Funding

AAA is supported by the National Institutes of Health (NIH) Cell and Molecular Training for Cardiovascular Biology Grant T32HL007208 awarded to the Cardiovascular Research Center at Massachusetts General Hospital. Research in the Burns Laboratory is supported by NIH Grants R01HL139806 (PI:CGB), R35HL135831 (PI:CEB), and R01HL127067 (MPI:CGB and CEB). CEB is a d’Arbeloff MGH Research Scholar, and CGB is an MGH Hassenfeld Cardiovascular Scholar.

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Authors and Affiliations

  1. Department of Cardiology, Boston Children’s Hospital, Boston, MA, 02115, USA

    Alexander A. Akerberg, Caroline E. Burns & C. Geoffrey Burns

  2. Cardiovascular Research Center, Massachusetts General Hospital, Charlestown, MA, 02129, USA

    Alexander A. Akerberg, Caroline E. Burns & C. Geoffrey Burns

  3. Harvard Medical School, Boston, MA, 02115, USA

    Alexander A. Akerberg, Caroline E. Burns & C. Geoffrey Burns

  4. Harvard Stem Cell Institute, Cambridge, MA, 02138, USA

    Caroline E. Burns

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  1. Alexander A. Akerberg
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Correspondence to C. Geoffrey Burns.

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Akerberg, A.A., Burns, C.E. & Burns, C.G. Exploring the Activities of RBPMS Proteins in Myocardial Biology. Pediatr Cardiol 40, 1410–1418 (2019). https://doi.org/10.1007/s00246-019-02180-6

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