MTG2 (mitochondrial ribosome associated GTPase 2) is a conserved GTPase that plays a critical role in mitochondrial ribosome maturation and quality control. As a member of the Obg family proteins, MTG2 functions primarily in the assembly of the large mitochondrial ribosomal subunit (mtLSU) 1. Mechanistically, MTG2 coordinates mtLSU maturation by facilitating 16S rRNA methylation through interaction with the methyltransferase MRM2, and serves as a quality control checkpoint by promoting the release of late-stage assembly factors to ensure proper formation of the mature 55S monosome 1. MTG2 also contacts the sarcin-ricin loop and immature active site during peptidyl transferase region assembly 2. Loss of MTG2 function leads to severely decreased 55S monosome levels and attenuated mitochondrial protein synthesis 1. Beyond mitochondrial translation, MTG2 has emerged as a disease-relevant target: it is associated with age-related macular degeneration (AMD) pathogenesis, with MTG2 expression significantly induced during retinal angiogenesis and subretinal fibrosis in murine AMD models 3. Additionally, MTG2 represents a causal and druggable target in uveal melanoma through modulation of mitochondrial DNA heteroplasmy and copy number, with dimercaptosuccinic acid identified as a potential therapeutic inhibitor 4. MTG2 also emerged in a survival-dependent gene signature for esophageal cancer prognosis 5.