Supplementary Materials

The PDF file includes:

  • Methods
  • Fig. S1. Glucose metabolism schema and survival curves of patients and mice with PDHD.
  • Fig. S2. Expression of PDH in the mouse somatosensory cortex.
  • Fig. S3. Phenotype of PDHD mice.
  • Fig. S4. Glucose metabolism in PDHD mice.
  • Fig. S5. EEG in patients with PDHD.
  • Fig. S6. Patient with PDHD EEG (patient I).
  • Fig. S7. EEG oscillation epochs in patients with PDHD.
  • Fig. S8. Cortical layering, evoked LFP, and baseline EEG in PDHD mice.
  • Fig. S9. Sorting of multiunit action potentials into single units and separation into broad- and fast-spiking neuronal origin.
  • Fig. S10. In vivo RS neuron discharge relative to fast-spiking activity.
  • Fig. S11. Brain slice intracellular recordings illustrating cell discrimination, rheobase, and firing.
  • Fig. S12. Neuronal input resistance under energetic substrate deprivation.
  • Fig. S13. EEG gamma power in relation to epileptiform events in PDHD.
  • Fig. S14. Gamma power in relation to epileptiform events in additional patients with PDHD.
  • Fig. S15. Acetate modulation of evoked LFPs in vivo.
  • Fig. S16. Acetate modulation of spontaneous excitatory postsynaptic currents.
  • Fig. S17. Modulation of input resistance and firing with acetate.
  • Fig. S18. Acetate modulation of EEG epileptiform events in awake mice.
  • Fig. S19. EEG epileptiform events in awake mice with vehicle injections.
  • Fig. S20. Representation of glucose oxidation and excitability coupling in relation to PDH activity.
  • Table S1. Plasma metabolite concentration.
  • Table S2. Isotopomer analysis of [U-13C]glucose NMR spectra from cerebral cortex NMR spectra.
  • Table S3. Isotopomers derived from [1,2-13C]acetate and [1,6-13C]glucose in cerebral cortex NMR spectra.
  • Table S4. Metabolic model analysis of glutamate and glutamine C4 multiplets from [1,6-13C]glucose and [1,2-13C]acetate cerebral cortex spectra.
  • Table S5. Modified Racine’s scale.
  • Table S6. Key clinical features of patients with PDHD.
  • Legend for table S7
  • Legend for movie S1
  • References (5775)

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Other Supplementary Material for this manuscript includes the following:

  • Table S7. Raw data (provided as a separate Excel file).
  • Movie S1 (.mp4 format). PDHD mouse video-EEG recording of epileptiform events.