As part of the MCIA complex, primarily participates in the assembly of the mitochondrial complex I and therefore plays a role in oxidative phosphorylation ( ). This moonlighting protein has also a dehydrogenase activity toward a broad range of substrates with greater specificity for long-chain unsaturated acyl-CoAs ( , ). However, in vivo, it does not seem to play a primary role in fatty acid oxidation (, ). In addition, the function in complex I assembly is independent of the dehydrogenase activity of the protein .
Subcellular locations: Mitochondrion inner membrane
Essentially associated with membranes.
Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver . In the cerebellum uniquely expressed in the granular layer (at protein level) .