MRP-L22 Inhibitors

MRP-L22, or Mitochondrial Ribosomal Protein L22, is a component of the large subunit (39S) of the mitochondrial ribosome in humans and is encoded by the MRPL22 gene. Mitochondrial ribosomes (mitoribosomes) are specialized for the synthesis of membrane proteins that are integral to the mitochondrial inner membrane and are involved in oxidative phosphorylation, the process by which cells generate ATP, the primary energy currency of the cell.The structure of mitoribosomes differs somewhat from their cytoplasmic counterparts, reflecting their specialized roles and the unique environment within mitochondria. MRP-L22 contributes to the structure and function of the 39S subunit, and although less is known about the individual roles of mitochondrial ribosomal proteins compared to their cytoplasmic counterparts, they are essential for mitochondrial protein synthesis. Mitochondrial ribosomal proteins, including MRP-L22, are nuclear-encoded and are imported into the mitochondria post-translation. Once inside the mitochondria, they are assembled into the ribosome. The mitochondrial genome encodes 13 proteins, all of which are components of the respiratory chain complexes and are synthesized within the mitochondria with the help of the mitoribosome.Mutations in genes encoding mitochondrial ribosomal proteins can lead to mitochondrial dysfunction and are associated with various human diseases. Diseases related to mitochondrial dysfunction can be multi-systemic due to the central role of mitochondria in energy production.