Mitochondrial ribosomal protein S14 (MRPS14)

Mitochondrial ribosomal protein S14 (MRPS14) is a nuclear-encoded protein that is part of the 28S small subunit of the mitochondrial ribosome. It is essential for mitochondrial protein synthesis by facilitating mRNA recruitment and translation elongation within the mitochondria. Diseases caused by pathogenic variants in MRPS14 include combined oxidative phosphorylation deficiency, hypertrophic cardiomyopathy, and lactic acidosis. Although mitoribosomal proteins differ substantially from their prokaryotic homologs, MRPS14 belongs to the ribosomal protein S14P family and performs a structural and functional role within the mitochondrial ribosome. MRPS14 is not a classical drug target, but the mitoribosome as a whole has been implicated as a target in the context of certain antibiotics and investigational cancer therapies. Disruption of MRPS14 impairs energy production and mitochondrial homeostasis, leading to severe clinical manifestations.

Other names

Small ribosomal subunit protein uS14mMRP-S14S14mtHSMRPS1428S ribosomal protein S14, mitochondrialCOXPD38DJ262D12.2mitochondrial small ribosomal subunit protein uS14mmitochondrial 28S ribosomal protein S14