InterPro domain: IPR018625

General Information

  • Identifier IPR018625
  • Description Protein Pet100
  • Number of genes 119
  • Gene duplication stats Loading...
  • Associated GO terms GO:0033617   GO:0005739  

Abstract

Pet100 is a chaperone required for the assembly of cytochrome c oxidase [ 1 ]. The human Pet100 homologue (also known as C19orf79) has been shown to be located in the mitochondrial inner membrane and forms a ~300kDa subcomplex with mitochondrial complex IV subunits [ 2 ].


1. Structure and function of Pet100p, a molecular chaperone required for the assembly of cytochrome c oxidase in Saccharomyces cerevisiae. Biochem. Soc. Trans. 29, 436-41
2. A founder mutation in PET100 causes isolated complex IV deficiency in Lebanese individuals with Leigh syndrome. Am. J. Hum. Genet. 94, 209-22

Species distribution

Gene table