OXA1L

This gene encodes an evolutionarily conserved protein that is localized to the inner mitochondrial membrane. The encoded protein is essential for the translocation of the N-terminal tail of subunit 2 of cytochrome c oxidase, and is involved in the assembly of the cytochrome c oxidase and ATPase complexes of the mitochondrial respiratory chain. [provided by RefSeq, Jul 2016]

OXA1L, Mitochondrial Inner Membrane Protein

Required for the insertion of integral membrane proteins into the mitochondrial inner membrane. Essential for the activity and assembly of cytochrome oxidase. Required for the correct biogenesis of ATP synthase and complex I in mitochondria.

Aerobic respirationManual Assertion Based On ExperimentIMP:UniProtKB
Mitochondrial cytochrome c oxidase assemblyManual Assertion Based On ExperimentIBA:GO_Central
Mitochondrial proton-transporting ATP synthase complex assemblyManual Assertion Based On ExperimentIMP:UniProtKB
Mitochondrial respiratory chain complex I assemblyManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of ATP-dependent activityManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of oxidoreductase activityManual Assertion Based On ExperimentIMP:UniProtKB
Protein insertion into membraneManual Assertion Based On ExperimentIBA:GO_Central
Protein insertion into mitochondrial inner membrane from matrixManual Assertion Based On ExperimentIBA:GO_Central
Protein tetramerizationManual Assertion Based On ExperimentIDA:UniProtKB

Mitochondrion inner membrane

Mitochondrial intermembrane: 1-113
Helical: 114-134
Mitochondrial matrix: 135-139
Helical: 140-160
Mitochondrial intermembrane: 161-212
Helical: 213-233
Mitochondrial matrix: 234-260
Helical: 261-281
Mitochondrial intermembrane: 282-299
Helical: 300-320
Mitochondrial matrix: 321-435