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Unravelling a role of LRPPRC in peroxisomal lipid metabolism through lipidomic investigations in human and mouse

LRPPRC (leucine‐rich pentatricopeptide repeat‐containing protein) is commonly described as a regulator of RNA metabolism in both mitochondria and nuclei, but the overall biological impact of this role remains unclear. Integrative genomics analyses have revealed that mutations in the LRPPRC gene were causing the cytochrome c oxidase defect in the French‐Canadian variant of Leigh Syndrome (LSFC) (Mootha et al. 2003). More recently, a targeted metabolomics study in LSFC patients highlighted specific markers reflecting mitochondrial metabolic perturbations for various nutrients including fatty acids (Thompson‐Legault et al. 2015). The role of LRPPRC in lipid metabolism remains, however, to be better deciphered.