By targeted proteomics we analyzed the content of several frataxin-related proteins in tissues collected from WT and FXNI151F mice. The proteins analyzed were two components of the tricarboxylic acids cycle (CS and Aco2), several components of the OXPHOS system (SDHA, SDHB, CY1, QCR2, COX2, ATPA, and ATPB), two mitochondrial chaperones (HSP60 and GRP75), three components of the Pyruvate dehydrogenase complex (PDHA1, DLAT and DLDH), and two superoxide dismutases (SOD1 and SOD2). Three glycolytic
...enzymes where also included in the analysis (GAPDH, PKM, and Enolases A, B, G). Two of the proteins analyzed contain iron-sulfur clusters (ACO2 and SDHB), while two of them (CYC and CY1) contain heme groups. The relative content of these proteins was analyzed in Brain, Cerebellum and Heart from WT and MUT mice, sacrificed at 21 or 39 weeks of age. Significant differences in the content of several proteins were observed between WT and MUT mice. The proteins more affected were ACO2 and the two components of OXPHOS complex II (SDHA and SDHB). These proteins showed a marked decrease in cerebellum and brain from MUT mice, both at 21 and 39 weeks. In heart, we could only observe loss of complex II in 21 weeks-old mice, but not in 39-week old mice, while ACO2 content was not altered in heart at any age.[Show more]