(VAR1) explained

Mitochondrial ribosomal proteins (MRPs) are essential components of the mitochondrial ribosome. Mitochondria are unique organelles in eukaryotic cells, often referred to as the “powerhouses” of the cell, responsible for producing ATP via oxidative phosphorylation. They have their own small genome and ribosomes, which are distinct from the cytosolic ribosomes.

Mitochondrial ribosomes (mitoribosomes) are responsible for synthesizing a small set of proteins that are essential components of the oxidative phosphorylation system. Given the bacterial ancestry of mitochondria, mitoribosomes have more in common with bacterial ribosomes than they do with eukaryotic cytosolic ribosomes, although there are differences from both.

There are two types of ribosomal subunits in the mitochondria:

1. Small subunit (28S or 12S in humans)
2. Large subunit (39S or 16S in humans)

Mitochondrial Ribosomes (mitoribosomes):

Mitochondrial ribosomes are responsible for the synthesis of 13 polypeptides that are components of the electron transport chain and ATP synthase, which are crucial for oxidative phosphorylation.

Differences between mitoribosomes and cytosolic ribosomes:

1. Size and Composition: While the eukaryotic cytosolic ribosomes are referred to as 80S ribosomes (composed of 60S and 40S subunits), mitoribosomes in humans are 55S, comprising 39S (large subunit) and 28S (small subunit). The ‘S’ here refers to the Svedberg unit, a measure of particle sedimentation rate during centrifugation, which indirectly indicates its size.
2. RNA Component: The rRNA in mitoribosomes is shorter than that in cytosolic ribosomes. For instance, human mitochondrial 12S and 16S rRNAs are counterparts of the bacterial 16S and 23S rRNAs, respectively. Despite being shorter, mitoribosomal RNAs (rRNAs) are heavily modified.
3. Protein Component: Mitoribosomes have a higher protein-to-RNA ratio than cytosolic ribosomes. This is because MRPs compensate for the shorter rRNA in mitochondria. Many MRPs have no counterparts in the bacterial or cytosolic ribosomes, making them unique.

MRPs and their role:

1. Diverse Origins: Some MRPs have clear bacterial ancestry, given the endosymbiotic theory which proposes that mitochondria evolved from a symbiotic relationship between an ancestral eukaryotic cell and an alpha-proteobacterium. Other MRPs have been recruited from the eukaryotic cytosol, and some are entirely novel with no known counterparts.
2. Functional Specialization: MRPs have roles beyond just the structural. They are involved in the unique aspects of mitochondrial translation, including specific mRNA recognition and interactions with the mitochondrial membrane.
3. Clinical Relevance: Defects in MRPs can lead to mitochondrial diseases. Given the critical role of mitochondria in energy production, defects in mitochondrial protein synthesis can manifest as a variety of clinical conditions, often affecting tissues with high energy demands like muscles and the nervous system.
4. Assembly: The assembly of the mitoribosome is a complex process that requires numerous assembly factors. This process ensures the correct maturation and incorporation of rRNA and MRPs.

In summary, mitochondrial ribosomal proteins play a pivotal role in the specialized protein synthesis machinery of the mitochondria. Research into MRPs not only sheds light on the fascinating evolution of mitochondria but also holds clinical relevance in understanding certain human diseases.