OXA1L is a mitochondrial inner membrane insertase that mediates the cotranslational insertion of integral membrane proteins into the mitochondrial inner membrane 1. The protein functions through three distinct contact sites with mitochondrial ribosomes, facilitating the delivery of newly synthesized polypeptides through conformational changes that create constriction sites in the ribosomal exit tunnel 1. OXA1L is essential for the biogenesis and assembly of key respiratory complexes, with knockdown studies demonstrating its requirement for F1Fo-ATP synthase and NADH:ubiquinone oxidoreductase (complex I) assembly and activity 2. The protein exists as part of a 600-700 kDa complex and its C-terminal tail binds to mitochondrial ribosomes with high affinity to enable cotranslational protein insertion 3. Clinically, OXA1L deficiency causes mitochondrial myopathy through combined respiratory chain defects and oxidative phosphorylation impairments 4. The pathogenic mechanism involves elevated reactive oxygen species production that activates nuclear factor kappa B signaling, leading to disturbed myogenic gene expression and cell apoptosis 4. Additionally, genetic variants in OXA1L have been associated with asthma and atopy in population studies 5.