Automated Behavioral Phenotyping Reveals Presymptomatic Alterations in a SCA3 Genetrap Mouse Model

Jeannette Hübener; Nicolas Casadei; Peter Teismann; Mathias W. Seeliger; Maria Björkqvist; Stephan von Hörsten; Olaf Riess; Huu Phuc Nguyen

doi:10.1016/j.jgg.2012.04.009

Volume 39 Issue 6

Jun. 2012

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Article Navigation > Journal of Genetics and Genomics > 2012 > 39(6): 287-299

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Automated Behavioral Phenotyping Reveals Presymptomatic Alterations in a SCA3 Genetrap Mouse Model

doi: 10.1016/j.jgg.2012.04.009

a
Department of Medical Genetics, University of Tübingen, Tübingen 72076, Germany
b
Institute of Medical Sciences, University of Aberdeen, AB25 2ZD, UK
c
Division of Ocular Neurodegeneration, Centre for Ophthalmology, Institute for Ophthalmic Research, University of Tübingen, Tübingen 72076, Germany
d
Neuronal Survival Unit, Department of Experimental Medical Sciences, Wallenberg Neuroscience Center, Lund University, Lund 221 84, Sweden
e
Experimental Therapy, Franz-Penzoldt-Center, University of Erlangen-Nürnberg, Erlangen 91054, Germany

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Corresponding author: E-mail address: jeannette.huebener@med.uni-tuebingen.de (Jeannette Hübener)
Received Date: 2012-03-19
Accepted Date: 2012-04-23
Rev Recd Date: 2012-04-18

Available Online: 2012-05-15

Publish Date: 2012-06-20

Abstract

Abstract

Characterization of disease models of neurodegenerative disorders requires a systematic and comprehensive phenotyping in a highly standardized manner. Therefore, automated high-resolution behavior test systems such as the homecage based LabMaster system are of particular interest. We demonstrate the power of the automated LabMaster system by discovering previously unrecognized features of a recently characterized atxn3 mutant mouse model. This model provided neurological symptoms including gait ataxia, tremor, weight loss and premature death at the age of 12 months usually detectable just 2 weeks before the mice died. Moreover, using the LabMaster system we were able to detect hypoactivity in presymptomatic mutant mice in the dark as well as light phase. Additionally, we analyzed inflammation, immunological and hematological parameters, which indicated a reduced immune defense in phenotypic mice. Here we demonstrate that a detailed characterization even of organ systems that are usually not affected in SCA3 is important for further studies of pathogenesis and required for the preclinical therapeutic studies.
- Automated homecage behavior,
- Genetrap mouse model,
- Spinocerebellar Ataxia Type 3

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References(38)

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