Rotarod testing provides a highly translational and reproducible behavioral readout, reflecting bona fide motor deficits that are characteristic of the AS phenotype. (A) The left panel presents pooled raw data from multiple independent experiments, highlighting a consistent reduction in motor performance compared to wild-type controls. (B) The right panel displays Forest plots of normalized data for individual experiments, where performance in each cohort is normalized to wild-type (represented by the dashed line) with 95% confidence intervals shown. These results underscore the robustness and reliability of Rotarod performance as a key outcome measure in preclinical studies using Ube3a mouse models. 

Figure obtained and re-formatted with permission from the original publication validating the behavioral phenotypes of Ube3a mutant mice (Sonzogni et al., 2018).

Assessments of audiogenic seizure susceptibility confirm and extend previous findings, establishing this phenotype as a powerful tool to investigate seizure vulnerability and potential therapeutic interventions in Ube3a mouse models. (A) The left panel presents audiogenic seizure susceptibility data for wild-type (WT) and Ube3a mice (n = 45, 114) on the 129S2/SvPasCrl background. (B) The right panel shows the effect of increasing doses of levetiracetam on seizure susceptibility in Ube3a mice, assessed by Fisher’s exact test. Together, these findings validate the audiogenic seizure paradigm as a sensitive and reproducible outcome measure in preclinical studies of Angelman syndrome.  

Figure obtained and re-formatted with permission from the original publication describing the validity and robustness of behavioral readouts for Ube3a mice based on a meta-analysis of 111 Ube3am−/p+ and 120 WT littermates on an F1 hybrid 129S2-C57BL/6J background (Sonzogni et al., 2018). See also FAQ below for more explanation on the background type used.