PMC

Survival of Hlf ^KO/+, Scn1a^KO/+ and Hlf ^KO/+;Scn1a^KO/+ mice was monitored until nine weeks of age. Kaplan Meier survival plot is shown. Hlf ^KO/+;Scn1a^KO/+ mice had reduced survival compared to Scn1a^KO/+ mice (p<0.006, LogRank Mantel-Cox).

A) Average FMS frequency of Q54 mice maintained on pyridoxine deficient or control diets from 3 to 9 weeks of age. The number of FMS was counted in a 30 minute session at 9 weeks of age. Significant differences were determined by Student’s T-test (*p < 0.03). Data are represented as mean ± SEM. B) Survival of Q54 mice maintained on pyridoxine deficient or control diets was monitored until nine weeks of age. Kaplan Meier survival plot is shown. Q54 mice on the deficient diet had a significant decrease in survival compared to control diet (p<0.002, LogRank Mantel-Cox).

A) Mice were observed for focal motor seizures (FMS) and generalized tonic clonic seizures (GTCS) in 30 minute sessions at 3 and 6 weeks of age. The percentages of mice exhibiting FMS or GTCS during the observation period are shown (n=30–32 per group). Significant differences between groups were determined by Fisher’s exact test. *p < 0.0134 compared to Q54 littermate controls. B) The average number of FMS (±S.E.M) and total number of GTCS observed is shown (n=30–32 per group). C) Kaplan-Meier plot of survival to 6 weeks of age. Hlf ^KO/KO;Q54 had a significant decrease in survival compared to Q54 mice (p=0.0215, LogRank Mantel-Cox). D) Representative EEG recording from a Hlf ^KO/KO;Q54 during a seizure. Time span of plot is 3 minutes. Top trace is right posterior to left posterior; bottom trace is right anterior to left posterior.