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MRPL17 encodes the 39S ribosomal protein L17, a component of the large subunit of the mammalian mitochondrial ribosome[1][5][7]. Unlike cytoplasmic ribosomal proteins, mitochondrial ribosomal proteins are encoded by nuclear genes, translated in the cytosol, and imported into mitochondria for assembly into the mitoribosome. MRPL17 is essential for the structural and functional integrity of the mitochondrial ribosome and for mitochondrial protein synthesis, which is critical for cellular energy production through oxidative phosphorylation[1][2][4][5]. Disruption of MRPL17 or other mitochondrial ribosomal proteins can result in impaired energy metabolism and is associated with various mitochondrial disorders and, in some cases, cancer[4]. Mammalian ribosomal proteins have low sequence conservation with bacterial homologs, underlining unique evolutionary modifications[1][4][6]. There is no redundancy among mitochondrial ribosomal proteins, and loss of function is usually lethal or results in severe disease phenotypes[4]. MRPL17 itself is not a therapeutic target and does not interact directly with drugs, but its function is indispensable for cell survival.
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