Mitochondrial ribosome-associated GTPase 1 (MTG1)

Mitochondrial ribosome-associated GTPase 1 (MTG1) is a conserved ribosome assembly GTPase critical for mitochondrial protein synthesis. In human mitochondria, MTG1 associates with the large ribosomal subunit in a GTP-dependent manner, where its GTPase activity is stimulated and facilitates incorporation of late-assembly ribosomal proteins. MTG1 acts as a quality control checkpoint, ensuring proper formation of intersubunit bridge during mitoribosome assembly. Depletion of MTG1 impairs mitochondrial translation and leads to defects in assembly/function of respiratory complexes, which in turn disrupts cellular energy production. Besides mitoribosome biogenesis, MTG1 is implicated in cardiac stress responses—helping to preserve respiratory complex activity and protecting against hypertrophy. Pathologically, recurrent MTG1-CYP2E1 fusions serve as molecular markers in certain benign mesenchymal tumors of the female lower genital tract[1][3][2].

Other names

GTPBP7GTP-binding protein 7Mitochondrial GTPase 1mitochondrial ribosome-associated GTPase 1mitochondrial GTPase 1 homolog