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. 2022 Aug 14;25(9):104941. doi: 10.1016/j.isci.2022.104941

Figure 4.

Figure 4

TRAP1 agonists ameliorate mitochondrial and ER stress

(A and B) ML405 decreases the red/green fluorescence ratio in Mitotimer-expressing NPC1 cells, indicating reduced mitochondrial oxidative stress (A, representative image of cell mitochondria quantified in B).

(C) Treatment with ML405/85 reduce superoxide levels (red; MitoSox) in isogenic wt and NPC1-null cells but have no effect in isogenic TRAP1-null cells, indicating that the reduction of mitochondrial superoxide is mediated by TRAP1.

(D) Treatment with ML405 increases ATP levels in both NPC3 and Fabry patient cells.

(E) Treatment with ML405 increases the residual α-galactosidase activity in Fabry patient cells to varying degrees based on the specific α-galactosidase mutation of each cell line.

(F–H) Similarly, treatment with ML405 increases the activity of lysosomal acid lipase in Wolman (F), tripeptidyl peptidase 1 in CLNII (G), and galactocerebrosidase in Krabbe (H) patient cells. Data are represented as the mean ± SD ∗∗∗p < 0.0005, ∗∗p < 0.001.