Figure 5.

KL1333 regulates intracellular ATP, lactate, and ROS levels in MELAS fibroblasts. Human fibroblasts derived from three patients with MELAS were treated with 1 μM KL1333 for 24 h. (A) Cells were lysed in extraction buffer, and intracellular ATP levels were measured. (B) Cell lysates were reacted with enzyme mixtures, and intracellular lactate levels were measured. (C) Cells were stained with CM-H2DCFDA, and ROS levels were measured using a flow cytometer. (D) MELAS 1 fibroblasts were treated with 1 μM KL1333 (KL) or 1 μM idebenone (Ide) for 24 h. Cell lysates were used for measurement of ATP levels. Each experiment was repeated three times. Error bars indicate ± SEM. ^#P < 0.05: MELAS fibroblast vs. WT control. ^*P < 0.05: presence versus absence of KL1333.