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. Author manuscript; available in PMC: 2015 Aug 5.
Published in final edited form as: Cell Metab. 2014 Jun 19;20(2):253–266. doi: 10.1016/j.cmet.2014.05.014

Figure 7. SDHB is destabilized in human cells with reduced levels of SDHAF1.

Figure 7

(A) Relative SDHAF1 mRNA levels in HEK293 cells 72 h after SDHAF1 knockdown using siRNA (left panel) and steady-state levels of proteins from total cell lysates (right panel). (B) HEK293 cells were treated with either control siRNA or SDHAF1 siRNA. Paraquat was added to cultures 24 h after siRNA transfection. Total cell lysates were obtained 48 h after paraquat treatment. (C) Steady-state levels of proteins in mitochondria isolated from control fibroblasts and patient fibroblasts harboring mutations on SDHAF1 (Ohlenbusch et al., 2012). The indicated percentages are relative levels of SDHB normalized to ATP5A levels by densitometry. (D) Model of the role of Sdh6(SDHAF1) and Sdh7(SDHAF3) in maturation of Sdh2(SDHB). Sdh6 and Sdh7 associate with Sdh2 within a Sdh1/Sdh2 intermediate. Sdh1 maturation requires covalent flavinylation by Sdh5 followed by formation of the Sdh1/Sdh2 subcomplex that is chaperoned by Sdh8 (see accompanying paper VanVraken et al. 2014).