The role of mitochondrial energy metabolism in maintaining lung function is not understood.
We previously observed reduced lung function in mice lacking the fatty acid oxidation
enzyme long-chain acyl-CoA dehydrogenase (LCAD). Here, we demonstrate that long-chain
acylcarnitines, a class of lipids secreted by mitochondria when metabolism is inhibited,
accumulate at the air-fluid interface in LCAD−/− lungs. Acylcarnitine accumulation is
exacerbated by stress such as influenza infection or by dietary supplementation with l …