Recombinant Human PET117 protein

Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

PET117 also known as PET117 homolog is a small protein with a molecular mass of approximately 9 kDa. It is expressed mainly in the mitochondria of cells. PET117 is associated with mitochondrial transport and assembly processes. As a mitochondrial protein it has a role in the complex systems that organize and regulate the energy production within the cell.

Biological function summary

PET117 facilitates the assembly of Complex IV (cytochrome c oxidase) within mitochondria. Complex IV is the terminal component of the electron transport chain playing an essential role in cellular respiration. PET117 acts in collaboration with other assembly factors and structural subunits to ensure proper assembly and function of Complex IV. Dysfunction in complex assembly affects overall mitochondrial efficiency and can lead to cellular energy deficits.

Pathways

PET117 is integral to oxidative phosphorylation which is the process by which cells generate ATP the primary energy currency. Within this pathway it connects closely with components of the electron transport chain like the Complex IV subunits. PET117 interacts directly or indirectly with proteins such as COX17 which is another factor critical for cytochrome c oxidase assembly.

Defects in PET117 have links to mitochondrial disorders specifically cytochrome c oxidase deficiency. This deficiency leads to a spectrum of neuromuscular diseases highlighting the importance of PET117 in maintaining mitochondrial function. PET117's connection to cytochrome c oxidase indicates its relevance in the pathology of these conditions as disruptions in the assembly process impact energy production negatively.