Figure 5.

Compensatory therapeutic approaches targeting autophagy reverts LHON cells’ predisposition to apoptotic death

(A and B) 11778 fibroblasts (A) and cybrids (B) harboring LHON mutations were treated with different autophagic inhibitors (3-MA [inhibitor of autophagy at early steps], chloroquine [CQ], and clozapine [CLOZ] [inhibitors of autophagy at late steps]). After 48 h, detection of autophagic activity through immunoblot technique was performed.

(C and D) Detection of apoptotic process activity on 11778 LHON fibroblasts (C) and cybrids (D) treated with anti-autophagic agents by immunoblotting with antibodies against PARP and CAS3 apoptotic markers.

(E and F) Cell viability in fibroblasts (E) and cybrids (F) pretreated with anti-autophagy compounds was performed at different time points.

Data are presented as means ± SEM. n = at least 3 independent experiments. ^∗p < 0.05, ^∗∗p < 0.01, and ^∗∗∗p < 0.001.