Updated project metadata. We analyzed the proteomic changes that occur in a Fbxl4 knock-out 1-year mouse. We analyzed Liver tissue using a 6-plex TMT approach (3 KO and 3 controls). Since we observed a global decrease in mitochondrial proteins, we also explored mitoproteome changes in different tissues (liver, kidney and heart) using a label-free approach. Finally, we also did a 6-plex TMT to analize the proteomic changes in 3 patient-derived fibroblast lines compared to 3 control lines and correlated them with the results obtained in the mouse model. All together, these experiments revealed that Fbxl4 deficiency leads to a decreased mitochondrial content without major changes in mitochondria itself, pointing to an increased turnover.