Fig. 5: OPA1-deficient NP cells evidence altered glucose and glutamine metabolism.

A Summation of flux results through glycolysis, pentose, and TCA cycle using [1,2]-¹³C-glucose and U¹³C-glutamine. B–G [1,2]-¹³C-glucose enrichment in the culture media from NP cells transduced with ShCtrl and ShOpa1 and cultured under hypoxia for 24 h. B Glycolysis flux as measured by enrichment of M2 lactate m/z 91, C Pentose cycle flux as measured by lactate (M1/M2)/ (3 + M1/M2) D PDH flux as measured glutamate m/z 103, E PC flux measured as glutamate m/z 104 F PC + PDH flux measured as glutamate m/z 131, G PDH/PC flux as measured by glutamate m/z 103-M1/104-M2. H–O [1,2]-¹³C-glucose tracing measured from the cell pellets into metabolites (H) alanine m/z 260, I serine m/z 390, J lactate m/z 261, K citrate m/z 591, L succinate m/z 289, M glutamate m/z 432, N palmitate (C16:0) m/z 313, O stearic acid (C18:0) m/z 341. P–W U¹³C-glutamine tracing measured from NP cells transduced with ShCtrl and ShOpa1 and cultured under hypoxia for 24 h P lactate m/z 261, Q citrate m/z 591, R succinate m/z 289, S fumarate m/z 287, T malate m/z 419, U aspartate m/z 418, V succinate oxidation and W fumarate reduction. Quantitative data are represented as box and whisker plots showing all data points with median and interquartile range and maximum and minimum values. Data points with negative values (undetectable ∑ mn) are not included. Statistical significance was computed using t-test or Mann-Whitney two-tailed test as appropriate. Source data are provided as a Source Data file.