Fig. 5: Comparative analysis of respiratory complexes I, II and III subunits containing FeS clusters in healthy and FRDA LCLs. | Cell Death & Disease

Fig. 5: Comparative analysis of respiratory complexes I, II and III subunits containing FeS clusters in healthy and FRDA LCLs.

From: Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain

Fig. 5: Comparative analysis of respiratory complexes I, II and III subunits containing FeS clusters in healthy and FRDA LCLs.The alternative text for this image may have been generated using AI.

Western blotting analysis of respiratory complexes subunits containing FeS clusters, i.e., NDUFS1, NDUFS8, NDUFS7, NDUFV1, NDUFV2 (complex I), SDHB (complex II) and UQCRFS1 (complex III), in whole cells extracts from FRDA (1 and 2) and healthy LCLs. Equal amounts of protein (i.e., 25 µg) were loaded in each lane. TOM20 was used as mitochondrial loading control. Protein levels were quantified after normalization with TOM20 and expressed as a percentage of control level (healthy LCLs). Reported data result from the mean of at least six independent experiments ± SEM. Statistical significance was determined using One-way ANOVA with Dunnett’s post-hoc test (*p ≤ 0.05, **p ≤ 0.01, compared to control). Figure reports the cartoon representation of human respiratory complex I structure (PDB ID: 5XTD) in which the subunits containing FeS clusters are highlighted in different colors (NDUFS1: light blue; NDUFS8: orange; NDUFS7: purple; NDUFV1: red; NDUFV2: pink). Iron and sulfur atoms belonging to FeS clusters are shown as brown and yellow spheres, respectively. To better visualize the number and position of FeS clusters, the subunits containing these cofactors were zoomed and reoriented on the right of the structural representation.

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