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Figure 4 | Orphanet Journal of Rare Diseases

Figure 4

From: Unfolded protein response in Gaucher disease: from human to Drosophila

Figure 4

UPR activation in Drosophila carriers of mutant GBA orthologs. A. GCase activity was tested in protein lysates prepared from either adult wild type (Canton S, WT) flies or from males and females of double heterozygous flies (Mi{ET1}CG31148,CG31414/CG31148,Mi{ET1}CG31414) using 4-MUG as a substrate. Presented are results of three experiments. B. RNA was isolated from double heterozygous flies, and the cDNA prepared from it was subjected to quantitative RT-PCR with primers specific for Drosophila Hsc-70-3 or for the spliced form of Drosophila Xbp1. The results (three different experiments) were quantified as explained in the legend to Figure 1A, and the values obtained for WT flies were considered 1. RP49 was used as a normalizing control. Dark box: WT flies; light box: double heterozygous flies. C. Protein lysates from double heterozygous flies (Double hets.) were subjected to western blotting and interaction with anti phosphorylated eIF2α antibodies (p-eIF2α). D. The results (three different experiments) were quantified as explained in the legend to Figure 2E and the values obtained for WT flies were considered 1. Significance: * < 0.05; ** < 0.01. E. The number of larvae, (L3,) that survived to the pupal stage, and the number of adults that eclosed was counted. The number of larvae taken for each experiment was 90.

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