Mitochondrial ribosomal protein L17

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Mitochondrial ribosomal protein L17 (MRPL17) is a protein that in humans is encoded by the MRPL17 gene. This protein is a component of the mitochondrial ribosome, which is specialized for the synthesis of mitochondrial proteins. The mitochondrial ribosome is distinct from the cytoplasmic ribosomes of the cell, and MRPL17 plays a critical role in the function of the mitochondrial ribosome, particularly in the assembly and stability of the large ribosomal subunit.

Function

MRPL17 is involved in the protein synthesis within the mitochondria, the energy-producing organelles of the cell. Mitochondrial ribosomes, also known as mitoribosomes, are responsible for synthesizing proteins that are essential for the mitochondrial electron transport chain, a series of reactions that generate adenosine triphosphate (ATP), the cell's main energy source. MRPL17, as part of the large subunit of the mitoribosome, contributes to the proper assembly and function of these ribosomes, ensuring efficient protein synthesis within the mitochondria.

Structure

The MRPL17 protein is one of the many ribosomal proteins that make up the mitochondrial ribosome. It is encoded by the MRPL17 gene located on the nuclear genome, but the protein itself is imported into the mitochondria after synthesis in the cytoplasm. The structure of MRPL17, like other mitochondrial ribosomal proteins, is adapted to function within the unique environment of the mitochondria, which has its own genetic code and machinery for protein synthesis.

Clinical Significance

Alterations in the MRPL17 gene or the MRPL17 protein function can lead to mitochondrial dysfunction, affecting the cell's energy production. This can contribute to a variety of mitochondrial diseases, which are a group of disorders caused by dysfunctional mitochondria. Symptoms of these diseases can range from muscle weakness and neurological disorders to systemic energy metabolism problems. Research into MRPL17 and other mitochondrial ribosomal proteins is crucial for understanding the molecular basis of these diseases and for developing potential therapeutic strategies.

See Also

• Mitochondrial DNA
• Ribosome
• Protein synthesis
• Mitochondrial disease