Fig. 5.

Metabolic fate of glucose and lactate in MNs derived from FUS–ALS patients and isogenic controls. a Fractional contribution of uniformly labelled ¹³C glucose (red) and uniformly labelled ¹³C lactate (blue) to metabolites from glycolysis and TCA cycle in patient (R521H or P525L) and isogenic control (R521R or P525P) motor neurons. b Relative abundance of each isotopologue for specific metabolites in patient and isogenic control motor neurons. The data represent mean ± s.e.m. from three independent experiments, with individual data points shown. Two-way ANOVA analysis with Tukey post hoc test was used to compare FUS-patients and isogenic controls, but did not show differences (p > 0.05). Dark grey bars indicate FUS-patient cell lines. Light grey bars indicate isogenic controls from these patients. Bar fill patterns show the corresponding patient and isogenic control. Thin black arrows represent direct metabolic pathway connections; dashed arrows indicate that additional intermediate reactions are involved in a metabolic pathway. Source data are provided as a source data file. ¹³C₆-glucose uniformly labelled glucose, ¹³C₃-lactate uniformly labelled lactate, PEP phosphoenolpyruvate, PC pyruvate carboxylase, PDH pyruvate dehydrogenase, αKG alpha-ketoglutarate, TCA tricarboxylic acid