Rapamycin rescues the bioenergetic profile of CSB-deficient cells. (A) Mitochondrial content of CS1AN cells expressing WT CSB (left) or an empty vector (right) was measured using flow cytometry and 50 nM MitoTracker green staining after treatment with the autophagy inducer 1 µM rapamycin (rapa), serum starvation (starve), or 10 mM of the autophagy inducer lithium chloride (LiCl; n = 3 representative experiments). (B) The membrane potential of CS1AN cells expressing WT CSB (left) or an empty vector (right) was measured using flow cytometry and 20 nM TMRM staining after treatment with 1 µM rapamycin (rapa) or 10 mM of the autophagy inhibitor 3-MA for 24 h (n = 6 representative experiments). (C) The bioenergetics profile of CS1AN cells expressing WT CSB or an empty vector after treatment with 10 mM lithium chloride, 1 µM rapamycin, or 3-MA for 24 h (n = 3; data are represented as mean ± SEM).