In collaboration with the Teleman lab (Heidelberg) the Vidal-Puig laboratory has identified a mechanism where by production of stearate by the enzyme Elovl6 is necessary to maintain appropriate mitochondrial function. Cells grown in media lacking stearate exhibited low mitochondrial respiration and fragmented mitochondria.
Stearate acts to prevent mitochondrial fragmentation by blocking Transferrin receptor 1 activity via stearoylation. This in turn prevents the phosphorylation and ubiquitination of mitofusin 2. Supplementing the diet of fly models of Parkinson’s disease was able to improve the life span of these models, suggesting stearate supplementation may be protective for diseases caused by mitochondrial defects.