title: Anxiety and risk assessment-related traits in a rat model of Spinocerebellar ataxia type 17.
abstract: Anxiety as a common feature of several neurodegenerative/polyglutamine diseases is an important aspect for the face validity of an animal model for Spinocerebellar Ataxia type 17 (SCA17). Risk assessment and anxiety-like traits were characterised in 3-6-9 months old rats of a transgenic model for SCA17 using the standard behavioural test elevated plus maze. In addition, c-Fos immunostainings in the basolateral amygdala evaluated neuronal activation in correlation to the behavioural responses. The most prominent behavioural effect was a higher level of risk assessment in the transgenic rats. In addition, an increase in anxiety-related behaviour in these rats was found. Although the EPM caused no overall effect on c-Fos expression, a negative correlation with the anxiety-like behavioural response was observed. Our results suggest that the SCA17 rat model displays an anxious phenotype already at 3 months of age resembling the generalized anxiety in early symptomatic SCA17 patients, thus confirming the validity of this rat model.
authors.affiliation: Department of Cognitive Neuroscience, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands; Noldus Information Technology BV, Wageningen, The Netherlands; Institute of Medical Genetics and Applied Genomics, University of Tübingen, 72076 Tübingen, Germany. Electronic address: eky@noldus.nl.
authors.forename: Elisavet I
authors.initials: EI
authors.lastname: Kyriakou
authors.affiliation: Department of Cognitive Neuroscience, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands; Noldus Information Technology BV, Wageningen, The Netherlands; Institute of Medical Genetics and Applied Genomics, University of Tübingen, 72076 Tübingen, Germany.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Manfré
authors.affiliation: Department of Cognitive Neuroscience, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors.forename: Jesús A
authors.initials: JA
authors.lastname: Spadaro
authors.affiliation: Institute of Medical Genetics and Applied Genomics, University of Tübingen, 72076 Tübingen, Germany; Centre for Rare Diseases, University of Tübingen, 72076 Tübingen, Germany. Electronic address: Hoa.Nguyen@med.uni-tuebingen.de.
authors.forename: Huu Phuc
authors.initials: HP
authors.lastname: Nguyen
authors.affiliation: Department of Cognitive Neuroscience, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands; Noldus Information Technology BV, Wageningen, The Netherlands.
authors.forename: Johanneke E Van der
authors.initials: JEV
authors.lastname: Harst
authors.affiliation: Department of Cognitive Neuroscience, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors.forename: Judith R
authors.initials: JR
authors.lastname: Homberg
mesh_terms: D000704:Analysis of Variance; D000818:Animals; D001007:Anxiety / Q000378:metabolism / Q000473:pathology; D066272:Basolateral Nuclear Complex / Q000378:metabolism / Q000473:pathology; D005106:Exploratory Behavior; D007150:Immunohistochemistry; D008297:Male; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D016760:Proto-Oncogene Proteins c-fos / Q000378:metabolism; D055647:Rats, Transgenic; D012309:Risk-Taking; D020754:Spinocerebellar Ataxias / Q000378:metabolism / Q000473:pathology / Q000523:psychology*
chemical_list: D016760:Proto-Oncogene Proteins c-fos
keywords: C-Fos neuronal activation; Disease development; Elevated plus maze; Model characterization; Polyglutamine disease