GTPBP10 is a GTPase involved in late-stage mitochondrial ribosomal large subunit (mtLSU) biogenesis 1. The protein associates with mtLSU precursors and functions as a ribosome maturation factor, forming complexes with other biogenesis factors including MALSU1, SMCR7L, MTERF4, and NSUN4 1. Structurally, GTPBP10 maintains immature conformations of 16S rRNA domains during assembly 2. GTPBP10 deficiency severely impairs 55S monosome formation, resulting in defective mitochondrial DNA expression and reduced cell growth 1. In disease contexts, GTPBP10 downregulation has been implicated in Huntington's disease progression, where edited microRNAs suppress GTPBP10 expression and contribute to mitochondrial dysfunction 3. Clinically, GTPBP10 exhibits differential expression across multiple cancer types and serves as a potential prognostic biomarker associated with immune microenvironment composition 4. Additionally, GTPBP10 shows cancer-associated alternative splicing patterns in leukemia 5. The protein coordinates with other GTPases like GTPBP5, GTPBP6, and GTPBP7 in ribosomal assembly pathways 67, suggesting functional interdependence among mitochondrial GTPases.