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  4. Comparative analyses of Purkinje cell gene expression profiles reveal shared molecular abnormalities in models of different polyglutamine diseases
 
research article

Comparative analyses of Purkinje cell gene expression profiles reveal shared molecular abnormalities in models of different polyglutamine diseases

Friedrich, Bernd
•
Euler, Philipp
•
Ziegler, Ruhtraut
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2012
Brain Research

Polyglutamine (PolyQ) diseases have common features that include progressive selective neurodegeneration and the formation of protein aggregates. There is growing evidence to suggest that critical nuclear events lead to transcriptional alterations in PolyQ diseases such as spinocerebellar ataxia type 7 (SCA7) and Huntington's disease (HD), conditions which share a cerebellar degenerative phenotype. Taking advantage of laser capture microdissection technique, we compared the Purkinje cell (PC) gene expression profiles of two transgenic polyQ mouse models (HD: R6/2; SCA7: P7E) by microarray analysis that was validated by real time quantitative PCR. A large number of transcriptional alterations were detected in the R6/2 transgenic model of HD. Similar decreases in the same mRNAs, such as phospholipase C, beta 3, purkinje cell protein 2 (Pcp2) and aldolase C, were found in both models. A decrease in aldolase C and phospholipase C, p 3, may lead to an increase in the vulnerability of PCs to excitotoxic events. Furthermore, downregulation of mRNAs mediated by the Pcp2-promoter is common in both models. Thus, our data reveal shared molecular abnormalities in different polyQ disorders. (C) 2012 Elsevier B.V. All rights reserved.

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Type
research article
DOI
10.1016/j.brainres.2012.08.005
Web of Science ID

WOS:000316711100003

Author(s)
Friedrich, Bernd
Euler, Philipp
Ziegler, Ruhtraut
Kuhn, Alexandre
Landwehrmeyer, Bernhard G.
Luthi-Carter, Ruth  
Weiller, Cornelius
Hellwig, Sabine
Zucker, Birgit
Date Issued

2012

Publisher

Elsevier Science Bv

Published in
Brain Research
Volume

1481

Start page

37

End page

48

Subjects

Huntington' s Disease

•

Spinocerebellar ataxia

•

Laser capture microdissection

•

Purldnje cell

•

Polyglutamine disease

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Microarray

Editorial or Peer reviewed

REVIEWED

Written at

EPFL

EPFL units
LNGF  
Available on Infoscience
May 13, 2013
Use this identifier to reference this record
https://infoscience.epfl.ch/handle/20.500.14299/92092
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