Abstract Mitochondria contain about 1,000–1,500 proteins that fulfil multiple functions.
Mitochondrial proteins originate from two genomes: mitochondrial and nuclear. Hence …

Mitochondria are essential organelles that execute and coordinate various metabolic
processes in the cell. Mitochondrial dysfunction severely affects cell fitness and contributes …

Across a variety of Mendelian disorders,∼ 50–75% of patients do not receive a genetic
diagnosis by exome sequencing indicating disease-causing variants in non-coding regions …

Mitochondria are essential metabolic hubs that dynamically adapt to physiological demands.
More than 40 proteases residing in different compartments of mitochondria, termed …

Mitochondrial ClpP is a serine protease located in the mitochondrial matrix. This protease
participates in mitochondrial protein quality control by degrading misfolded or damaged …

Regulation of the turnover of complex I (CI), the largest mitochondrial respiratory chain
complex, remains enigmatic despite huge advancement in understanding its structure and …

The mitochondrial ribosome (mitoribosome) is a multicomponent machine that has unique
structural features. Biogenesis of the human mitoribosome includes correct maturation and …

The caseinolytic protease proteolytic subunit (ClpP) is a serine protease playing an
important role in proteostasis of eukaryotic organelles and prokaryotic cells. Alteration of …

In prokaryotic cells and eukaryotic organelles, the ClpP protease plays an important role in
proteostasis. The disruption of the ClpP function has been shown to influence the infectivity …

Mitochondria control life and death in eukaryotic cells. Harboring a unique circular genome,
a by‐product of an ancient endosymbiotic event, mitochondria maintains a specialized and …