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Mitochondrial ribosomal protein L39 (MRPL39) is a nuclear gene-encoded protein that forms part of the large 39S subunit of the mitochondrial ribosome (mitoribosome), which is responsible for protein synthesis within the mitochondria[1][3][5][9]. MRPL39 is essential for the translation of mitochondrial genome-encoded proteins required for oxidative phosphorylation and cellular energy production[3][5][10]. Deficiency or biallelic variants in the MRPL39 gene cause impaired mitochondrial translation, leading to conditions such as combined oxidative phosphorylation deficiency 59 and other mitochondrial diseases presenting with multisystem symptoms[5][12]. MRPL39 is not currently considered a direct therapeutic target, receptor, or typical drug target class[5][12].
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