GTP-binding protein 8 (GTPBP8)

GTP-binding protein 8 (GTPBP8) is a nuclear-encoded GTPase localized to the mitochondrial matrix, peripherally associated with the inner mitochondrial membrane[2]. It plays a critical role in the late-stage assembly of the mitochondrial ribosome large subunit (mt-LSU), interacting specifically with the mitochondrial 16S rRNA, and is essential for proper mitoribosome biogenesis[1][2][3]. Loss of GTPBP8 results in accumulation of assembly intermediates, disrupted formation of functional monosomes, impaired mitochondrial translation, and a marked defect in oxidative phosphorylation[1][2][6]. While vital for mitochondrial gene expression and cellular energy metabolism, GTPBP8 is not currently considered a therapeutic drug target, nor are there known drugs, biomarkers, or established safety concerns directly associated with this protein[3][5]. Its dysfunction could theoretically contribute to human mitochondrial disorders, but concrete links to specific diseases are still under investigation[4].

Other names

HSPC135GTP binding protein 8 (putative)GTPBP8

Not applicable; no known drugs target GTPBP8