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

Research output: Contribution to journalArticlepeer-review

12 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)287-999
Number of pages13
JournalJournal of genetics and genomics
Issue number6
Publication statusPublished - 20 Jun 2012

Bibliographical note

Copyright © 2012. Published by Elsevier Ltd.


  • age factors
  • inborn errors
  • animals
  • behavior, animal
  • disease models, animal
  • Machado-Joseph disease
  • male
  • mice
  • mice, transgenic
  • microglia
  • mutation
  • nuclear proteins
  • phenotype
  • transcription factors
  • amino acid metabolism


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