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Mitochondrial ribosomes consist of a large 39S subunit and a small 28S subunit, both of which are comprised of multiple mitochondrial ribosomal proteins (MRPs) that are encoded by nuclear genes and are essential for protein synthesis within mitochondria. MRP-L32 (mitochondrial ribosomal protein L32), also known as HSPC283, is a 188 amino acid protein that localizes to the mitochondrion, where it exists as a component of the 39S ribosomal subunit and works in conjunction with other MRPs to mediate protein synthesis. The gene encoding MRP-L32 maps to human chromosome 7, which houses over 1,000 genes and comprises nearly 5% of the human genome. Defects in genes localized to chromosome 7 have been linked to Osteogenesis imperfecta, Williams-Beuren syndrome, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome.
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