ATP23 metallopeptidase and ATP synthase assembly factor homolog (ATP23)

ATP23 metallopeptidase and ATP synthase assembly factor homolog is a mitochondrial metallopeptidase and chaperone located in the intermembrane space of mitochondria. It plays a dual role: (1) processing the mitochondrial-encoded ATP synthase subunit Atp6 by cleaving its N-terminal presequence, and (2) promoting the assembly of mature Atp6 into the F1FO-ATP synthase complex independently of its proteolytic function. ATP23 contains a conserved metal-binding motif indicative of metallopeptidase activity. Although essential for F1FO-ATP synthase biogenesis in yeast, some functional differences exist in higher organisms; the human homolog is highly conserved but less is known about its processing targets. ATP23 is not a canonical therapeutic target but participates in mitochondrial energy metabolism and its gene is amplified in some cancers, such as glioblastoma

Other names

Mitochondrial inner membrane protease ATP23 homologKUB3XRCC6BP1Ku70-binding protein 3XRCC6-binding protein 1