Mitochondrial inner membrane protein OXA1L (OXA1L)

Mitochondrial inner membrane protein OXA1L (OXA1L) is an evolutionarily conserved membrane protein insertase essential for integrating both mitochondrially and nuclear-encoded proteins into the mitochondrial inner membrane. The protein facilitates the co-translational insertion of substrates by directly interacting with the mitochondrial ribosome—particularly via its C-terminal tail—which forms a conduit from the ribosomal exit tunnel to the membrane, controlling the folding and delivery of nascent polypeptides[1][2][3][4]. OXA1L is critical for the assembly of key components of the mitochondrial respiratory chain—including complexes I, IV, and V—and disruptions in its function cause OXPHOS deficiency and severe mitochondrial disease (such as encephalopathy)[2][5]. Accessory factors such as TMEM126A modulate OXA1L function and participate in quality control to clear failed insertion complexes from the inner membrane[2]. While OXA1L is mechanistically vital for mitochondrial function and disorders have been linked to OXA1L mutations, it is not currently a recognized direct therapeutic target or a site of drug action[1][2][4].

Other names

OXA1OXA1-like proteinOXA1L1OXA1Hsoxidase (cytochrome c) assembly 1-like proteinmitochondrial inner membrane insertaseMGC133129