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Abstract

Behavior

Behavioral Characterization of an Angelman Syndrome Mouse Model

Published: October 20th, 2023

DOI:

10.3791/65182

1Czech Centre for Phenogenomics, Institute of Molecular Genetics of the Czech Academy of Sciences, 2Laboratory of Transgenic Models of Diseases, Institute of Molecular Genetics of the Czech Academy of Sciences

Abstract

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.

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Keywords Angelman Syndrome

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