Proteomics

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Analysis of proteomic changes in Fbxl4 knock-out mouse liver and patient fibroblasts

ABSTRACT: 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.

INSTRUMENT(S): Orbitrap Fusion, Q Exactive HF

ORGANISM(S): Homo Sapiens (human) Mus Musculus (mouse)

TISSUE(S): Heart, Liver, Cell Culture, Fibroblast, Kidney

SUBMITTER: David Alsina  

LAB HEAD: Nils-Göran Larsson

PROVIDER: PXD018639 | Pride | 2020-06-10

REPOSITORIES: Pride

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Pathogenic variants in FBXL4 cause a severe encephalopathic syndrome associated with mtDNA depletion and deficient oxidative phosphorylation. To gain further insight into the enigmatic pathophysiology caused by FBXL4 deficiency, we generated homozygous Fbxl4 knockout mice and found that they display a predominant perinatal lethality. Surprisingly, the few surviving animals are apparently normal until the age of 8-12 months when they gradually develop signs of mitochondrial dysfunction and weight  ...[more]

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