Mitochondrial ribosomal protein S34 (MRPS34)

Mitochondrial ribosomal protein S34 (MRPS34) is a nuclear-encoded protein and a structural constituent of the mitochondrial small (28S) ribosomal subunit. It is essential for mitochondrial protein synthesis by facilitating the stability of the small ribosomal subunit and the 12S rRNA, both required for efficient mitoribosome formation and maintenance. Mutations in MRPS34 disrupt mitochondrial translation, compromise the stability of the 12S rRNA, reduce mitochondrial respiratory complex activity, and result in mitochondrial dysfunction and inherited disorders such as combined oxidative phosphorylation deficiency 32 and Leigh syndrome. MRPS34 does not act as a classical drug target (e.g., enzyme or receptor) but is instead classified as a ribosomal structural protein involved in mitochondrial gene expression and energy production, and disruptions to its function have direct clinical relevance in mitochondrial pathologies.

Other names

Small ribosomal subunit protein mS34MRP-S34S34mtMRP-S12MGC2616mS3428S ribosomal protein S34, mitochondrialCOXPD32MRPS12mitochondrial 28S ribosomal protein S34mitochondrial small ribosomal subunit protein mS34MRPS34phMRPS34C9JJ19P82930