Skip to main content
. 2020 Feb 6;87(3):339–346. doi: 10.1002/ana.25676

Figure 2.

Figure 2

Scn8a antisense oligonucleotide (ASO) delays seizure onset and prolongs survival of mutant mice expressing the pathogenic mutation SCN8A‐R1872W. (A) Scn8a cond/+, EIIA‐CRE mice were treated on postnatal day 2 by intracerebroventricular injection with the indicated mount of control or Scn8a ASO. A second dose of 100μg of Scn8a ASO at postnatal day 35 further extended survival (p < 0.001) (dotted line). (B) Dose dependence of mean survival; values are mean ± standard error of the mean. (C) Scn8a cond/+, EIIA‐CRE mice treated with 45μg exhibit normal posture without hind limb clasping at P21. (D) Duration of the effect of Scn8a ASO on transcript level. Brain RNA was prepared from Scn8a cond/+, EIIA‐CRE mice treated with 45μg of ASO and untreated age‐matched wild‐type mice and analyzed by quantitative reverse transcription polymerase chain reaction. (E) Reduction of Nav1.6 protein in brain at 3 weeks of age in mice treated with 45μg of ASO as in (D).