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. 2016 Jan 11;6:19118. doi: 10.1038/srep19118

Figure 10. VHL interacts with ALS-linked SOD1 mutants, and promotes the aggregation.

Figure 10

(a) Interaction of VHL with ALS-linked mutant SOD1 proteins. Lysates from HEK293A cells transfected with HA-VHL and WT or ALS-linked mutant SOD1-FLAG (A4V, H46R, G85R, and G93A) were immunoprecipitated with anti-FLAG affinity beads and analyzed by Western blotting using anti-HA antibody. (b) At 48 h after transfection of SOD1-FLAG (WT or G93A mutant) and/or HA-VHL, cells were fixed and stained as shown in Fig. 1. The number of cells containing SOD1-positive aggregates were counted (see supplementary Figure S10). VHL promoted the formation of SOD1 aggregates. Data are the average from three independent experiments. Differences were evaluated by one-way ANOVA (mean ± SD; *p < 0.05). n.s. indicates not significant (p = 0.0533). (c,d) Western blot analysis of VHL, CUL2 and HIF1α in the spinal cord lysates from mutant SOD1 transgenic mice. The tissue lysates from spinal cords of Tg mice of 280 days old, and non-transgenic mice of 230 days old (three mice each), were analyzed by blot images (c) and densitometry 8 d). The difference between the non Tg and G93A Tg in each protein was assessed by Stuent’s t test. n.s. indicates not significant.