Behavioral Characterization of an Angelman Syndrome Mouse Model

0581626 - ÚMG 2024 RIV US eng J - Journal Article
Kubik-Zahorodna, Agnieszka - Procházka, Jan - Sedláček, Radislav
Behavioral Characterization of an Angelman Syndrome Mouse Model.
Jove-Journal of Visualized Experiments. Roč. 2023, č. 200 (2023), č. článku e65182. ISSN 1940-087X
R&D Projects: GA MŠMT(CZ) LM2018126; GA MŠMT EF16_013/0001789; GA MŠMT EF18_046/0015861; GA MŠMT ED2.1.00/19.0395; GA MŠMT LM2023036
Institutional support: RVO:68378050
Keywords : Angelman Syndrome * Animals * Behavior * Animal * Disease Models
OECD category: Biochemistry and molecular biology
Impact factor: 1.2, year: 2022
Method of publishing: Limited access
https://www.jove.com/t/65182/behavioral-characterization-of-an-angelman-syndrome-mouse-model

This manuscript describes a battery of behavioral tests available to characterize Angelman syndrome (AS)-like phenotypes in an established murine model of AS. We use the rotarod learning paradigm, detailed gait analysis, and nest building test to detect and characterize animal motor impairments. We test animal emotionality in the open field and elevated plus maze tests, as well as the affect in the tail suspension test. When AS mice are tested in the open field test, the results should be interpreted with care, since motor dysfunctions influence mouse behavior in the maze and alter activity scores. The reproducibility and effectiveness of the presented behavioral tests has already been validated in several independent Uba3a mouse lines with different knockout variants, establishing this set of tests as an excellent validation tool in AS research. Models with the relevant construct and face validity will warrant further investigations to elucidate the pathophysiology of the disease and grant the development of causal treatments.
Permanent Link: https://hdl.handle.net/11104/0349879