RBM27 is an RNA-binding protein functioning as a critical regulator of nuclear RNA metabolism and neurodevelopmental processes. Mechanistically, RBM27 operates within the PAXT (Poly(A) Tail eXosome Targeting) complex, a nuclear RNA degradation pathway 1. It associates with core PAXT components including MTR4, ZFC3H1, and PABPN1 to facilitate recruitment of the RNA exosome to polyadenylated RNA substrates 12. RBM27 also participates in pre-mRNA splicing regulation, where PABPN1 recruits RBM26/27 to promote terminal intron splicing through interactions with the coiled-coil and RRM domains of RBM27 3. Beyond RNA processing, RBM27's ortholog RBM-26 in C. elegans negatively regulates expression of MALS-1, a mitoribosomal assembly factor, protecting against mitochondrial dysfunction and axon degeneration during neurodevelopment 4. Autism-associated missense variants in RBM27 reduce protein expression and cause axonal defects through dysregulation of MALS-1 4, suggesting RBM27's relevance to neurodevelopmental disorders. In cancer contexts, RBM27 is upregulated in glioblastoma and associates with altered immune microenvironment composition 5, and alternative RBM27 isoforms are detected in hepatocellular carcinoma 6. These findings establish RBM27 as a multifunctional RNA regulator with implications for both normal development and disease pathogenesis.