Rbpms2, an RNA-binding protein, is a member of the RNA-recognition-motif-containing protein family. It is involved in multiple biological processes such as alternative splicing regulation, sexual differentiation, and early embryo development. In the heart, it is a myocardial-enriched splicing regulator crucial for cardiac function. In sexual development, it promotes female fate and is related to pathways like mTorc1 signaling. Genetic models like knockout zebrafish and mice are valuable for studying its functions [1,2,3,5,6].

In zebrafish, rbpms2-deficient embryos have early cardiac dysfunction, myofibril disarray, and altered calcium handling, along with differential alternative splicing events. These phenotypes are also seen in RBPMS2-deficient human cardiomyocytes, uncovering a conserved network of 29 ortholog pairs regulated by RBPMS2 for alternative splicing [1]. In zebrafish gonads, rbpms2 mutants initially have early oocytes but definitive oogenesis fails during sexual differentiation, developing as fertile males. Rbpms2 promotes nucleolar amplification via the mTorc1 signaling pathway through the Gator2 component Mios [2,3,4]. In mouse models, Rbpms and Rbpms2 double cardiomyocyte-specific knockout mice die before embryonic day 13.5 with sarcomere disarray due to widespread mis-splicing of genes essential for cardiac contraction, while single knockout mice survive to adulthood [5].

In summary, Rbpms2 is essential for cardiac function, oogenesis, and sexual differentiation. Studies using gene knockout models in zebrafish and mice have revealed its role in regulating alternative splicing in the heart, and its function in promoting female fate in gonads. These findings contribute to understanding cardiovascular diseases and reproductive disorders [1,2,3,4,5].