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

Huebener, Jeannette; Casadei, Nicolas; Teismann, Peter; Seeliger, Mathias W.; Björkqvist, Maria LU ; von Hoersten, Stephan; Riess, Olaf and Nguyen, Huu Phuc (2012) In Journal of Genetics and Genomics 39(6). p.287-299
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... (More)
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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Automated homecage behavior, Genetrap mouse model, Spinocerebellar, Ataxia Type 3
in
Journal of Genetics and Genomics
volume
39
issue
6
pages
287 - 299
publisher
Science Press
external identifiers
  • wos:000305958500009
  • scopus:84863098979
ISSN
1673-8527
DOI
10.1016/j.jgg.2012.04.009
language
English
LU publication?
yes
id
e99269fc-854f-486e-92f5-905273092c92 (old id 3001783)
date added to LUP
2012-09-03 07:20:47
date last changed
2017-01-01 03:38:04
@article{e99269fc-854f-486e-92f5-905273092c92,
  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.},
  author       = {Huebener, Jeannette and Casadei, Nicolas and Teismann, Peter and Seeliger, Mathias W. and Björkqvist, Maria and von Hoersten, Stephan and Riess, Olaf and Nguyen, Huu Phuc},
  issn         = {1673-8527},
  keyword      = {Automated homecage behavior,Genetrap mouse model,Spinocerebellar,Ataxia Type 3},
  language     = {eng},
  number       = {6},
  pages        = {287--299},
  publisher    = {Science Press},
  series       = {Journal of Genetics and Genomics},
  title        = {Automated Behavioral Phenotyping Reveals Presymptomatic Alterations in a SCA3 Genetrap Mouse Model},
  url          = {http://dx.doi.org/10.1016/j.jgg.2012.04.009},
  volume       = {39},
  year         = {2012},
}