PQBP1 is an intrinsically disordered X-linked protein functioning as a multifaceted cellular regulator involved in pre-mRNA splicing, transcription, translation, and innate immunity 1. As a splicing factor, PQBP1 interacts with spliceosome components to regulate alternative splicing of target genes, including those controlling apoptosis and neural development 23. PQBP1 promotes translational elongation by suppressing eEF2K-mediated phosphorylation of eEF2, thereby regulating hippocampal synaptic plasticity and cognitive function 4. In innate immunity, PQBP1 acts as a pathogen-recognition receptor detecting retroviral reverse-transcribed DNA and other viral products through direct binding, subsequently activating the cGAS-STING signaling pathway to trigger type-I interferon production 56. PQBP1 also senses pathogenic tau protein to activate microglial inflammation via cGAS-STING, linking viral and neurodegenerative pathways 7. Mutations in PQBP1 cause Renpenning syndrome, characterized by intellectual disability, microcephaly, and developmental abnormalities 18. PQBP1 dysregulation also promotes ovarian cancer progression by suppressing pro-apoptotic BAX expression through aberrant splicing 2. These diverse functions underscore PQBP1's importance in neural development, immune defense, and cancer biology.