Mitochondria are the organelles that extract energy from nutrients, and they have their own tiny genomes. The DNA in mitochondria is important for energy generation. Generally, it’s thought that mitochondria were originally independent bacteria that harbored the ability to use highly toxic oxygen molecules as a energy source. Eukaryotes swallowed these bacteria but did not digest it, and then obtained the same ability to utilize oxygen molecules. Correct mitochondrial function is essential to the health of the bodies. When mitochondria fail to function properly, diseases can occur. Understanding the role of each component of mitochondria will provide a better map of mitochondrial function and help uncover the causes of mitochondrial diseases. Most human cells contain mitochondria. These small organelles generate adenosine triphosphate (ATP) for cells. One in 4,000 people will be affected by certain forms of mitochondrial diseases, many of which remain poorly understood. Current treatment options for mitochondrial diseases are limited. Scientists studying proteins associated with mitochondria have identified the functions of three proteins related to disease. The two studies, led by Dave Pagliarini, have been reported in Molecular Cell. Related proteins like Recombinant Mmel1 are offered by CusAb. Pagliarini and colleagues worked to explore the functions of more than 200 mitochondria-related proteins, whose roles remain to be defined. To accomplish this, Pagliarini’s team set out to investigate the interaction between these mitochondria-related proteins and other proteins. The study was carried out in collaboration with Josh Coon's laboratory. Using an advanced technique called affinity enrichment mass spectrometry, the researchers identified the function of three previously unknown proteins. They found that the C17orf89 protein plays a crucial role in the assembly of complex I, a type of protein that is involved in ATP generation. Further, they discovered that the LYRM5 protein interacts with electron-transferring flavoprotein. Finally, they uncovered that proteins involved in coenzyme Q biosynthesis form a dynamic “complex Q”. The study could help handle various mitochondrial diseases. Due to the important biological role of mitochondria, mitochondria dysfunction is associated with many diseases. The current research shows the functions the certain proteins related to mitochondria, a discovery that would provide new insight into mitochondrial protein function.