Mitochondrial ribosomal protein L18

Mitochondrial ribosomal protein L18 (MRPL18) is a protein that in humans is encoded by the MRPL18 gene. This protein is a component of the mitochondrial ribosome, which is specialized for the synthesis of mitochondrial proteins. Mitochondrial ribosomes, or mitoribosomes, are crucial for the production of proteins that are essential for the mitochondrion's structure and function. The role of MRPL18, alongside other mitochondrial ribosomal proteins, is pivotal in the process of protein synthesis within the mitochondria, which is key to cellular respiration and energy production.

Function

MRPL18 is part of the large subunit of the mitochondrial ribosome. Mitochondrial ribosomes are responsible for synthesizing proteins that are encoded by the mitochondrial DNA (mtDNA), which are primarily involved in the electron transport chain and oxidative phosphorylation (OXPHOS) processes. These processes are critical for the generation of adenosine triphosphate (ATP), the cell's main energy source. The proper assembly and function of mitochondrial ribosomes are essential for the maintenance of mitochondrial health and function, which in turn supports cellular energy needs and prevents mitochondrial diseases.

Gene

The MRPL18 gene is located on the nuclear genome and encodes the MRPL18 protein. After synthesis in the cytoplasm, MRPL18 is imported into the mitochondria, where it becomes part of the mitochondrial ribosome. The regulation of the MRPL18 gene expression is crucial for the proper synthesis of mitochondrial proteins and the overall function of the mitochondrion.

Clinical Significance

Alterations in the MRPL18 gene or its protein product can lead to mitochondrial dysfunction, which is associated with a range of mitochondrial diseases, including mitochondrial myopathy, neurodegenerative diseases, and other conditions characterized by impaired energy production. Understanding the role of MRPL18 in mitochondrial function helps in the study of these diseases and the development of potential therapeutic strategies.

See Also

• Mitochondrial DNA
• Mitochondrial disease
• Protein synthesis
• Ribosome