Mitochondrial ribosomal protein L11

Mitochondrial ribosomal protein L11 (MRPL11) is a protein that in humans is encoded by the MRPL11 gene. This protein is a component of the mitochondrial ribosome, which is specialized for the synthesis of mitochondrial proteins. Mitochondrial ribosomes, also known as mitoribosomes, are crucial for the production of proteins that are essential for the mitochondrion's structure and function.

Function

Mitochondrial ribosomal protein L11 plays a critical role in the assembly and function of the mitochondrial ribosome. It is involved in the synthesis of mitochondrial DNA-encoded proteins, which are primarily components of the electron transport chain and ATP synthase. These proteins are vital for oxidative phosphorylation, a process that generates adenosine triphosphate (ATP), the cell's main energy currency. By participating in the production of these proteins, MRPL11 helps maintain mitochondrial energy production and overall cellular energy homeostasis.

Gene

The MRPL11 gene is located on the human chromosome 19. It contains several exons and introns that are spliced together to produce the final mRNA, which is then translated into the MRPL11 protein. The regulation of this gene's expression is coordinated with the demand for mitochondrial protein synthesis, which can vary depending on the cell's metabolic state and energy needs.

Structure

As a component of the mitochondrial ribosome, MRPL11 contributes to the ribosome's large subunit. The structure of mitochondrial ribosomes differs significantly from their cytoplasmic counterparts, reflecting their specialized roles in the mitochondrion. MRPL11, like other mitochondrial ribosomal proteins, is encoded by nuclear DNA, synthesized in the cytoplasm, and then imported into the mitochondrion.

Clinical Significance

Alterations in the MRPL11 gene or the MRPL11 protein function can lead to mitochondrial dysfunction, which is associated with a range of human diseases. Mitochondrial disorders often affect tissues with high energy demands such as the brain, heart, and muscles, leading to complex clinical syndromes. However, the specific role of MRPL11 mutations in human disease remains an area of ongoing research.

See Also

• Mitochondrial DNA
• Oxidative phosphorylation
• Electron transport chain
• Mitochondrial disease