Fig. 1.

Higher contribution of exogenous glucose to pyruvate formation in Leigh syndrome French Canadian type (LSFC) fibroblasts. A and B: immunoblot and densitometric analysis of leucine-rich pentatricopeptide repeat-containing (LRPPRC, n = 12; A) and cytochrome-c oxidase subunit IV (COX IV, n = 9; B) protein levels in primary fibroblasts from LSFC and control donors. C and D: immortalized control and LSFC fibroblasts were incubated with 5 mM [U-¹³C₆]glucose, 0.2 mM pyruvate, and 0.5 mM glutamine for 4 h in serum-free DMEM, and the relative contribution of glucose to the formation of pyruvate, lactate, and alanine was analyzed by GC-MS (n = 7). Results represent means ± SE. Difference between control and LSFC cells was assessed with a paired Student’s t-test. *P ≤ 0.05; ***P ≤ 0.001.