Seizure and redox rescue in a model of glucose transport deficiency
Fig 5
The seizure phenotype in the GLUT1-DS model results from after-discharge firing, and can be rescued by either GLC or ATP.
a) time series of ATP in the neuron (left panel) and voltage traces (right panel) during control (green), GLUT1-DS (orange), glc (purple), glc-lac-bHb (blue), and bHB-lac-NAD-Q therapy (purple). Note the saw-tooth pattern in the afterdischarge trace, which corresponds to neuronal firing patterns, is eliminated by glc and glc-lac-bHb therapy. b) separated individual voltage traces from panel a, right, for clarity. c) time series of ATP in the neuron (left panel) and voltage traces (right panel) for control, GLUT1-DS and 5 doses of ATP. d) separated individual voltage traces from panel c, right, for clarity. Note that as the dose of ATP increases, the number of afterdischarge APs reduces until the seizures are completely gone at dose 4. Gray shaded area indicates time of neuronal stimulation.