Protein PET100 homolog, mitochondrial (PET100)

Protein PET100 homolog, mitochondrial (PET100) is a highly conserved mitochondrial chaperone essential for the assembly and stabilization of cytochrome c oxidase (complex IV) of the mitochondrial respiratory chain[1][2][3][5][7]. Localized to the inner mitochondrial membrane and exposed to the intermembrane space, PET100 acts as an assembly factor that coordinates integration of core subunits and ancillary proteins, working together with PET117 and MR-1S to ensure effective complex IV formation. Loss-of-function mutations in PET100 disrupt assembly of complex IV, causing severe mitochondrial disorders such as Leigh syndrome and fatal infantile lactic acidosis, with features of neurodegeneration and impaired energy production[1][5][7][9]. Currently, PET100 is not considered a direct therapeutic target but is important as a disease gene whose mutation results in profound deficiency of mitochondrial electron transport and cellular respiration.

Other names

PET100 cytochrome c oxidase chaperoneC19orf79MC4DN12PET100 homologPET100 mitochondrialprotein PET100 homolog, mitochondrial

PET100 is not known to be a direct target of drug interventions—it is a chaperone required for cytochrome c oxidase maturation[1][7][9].