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Euploidy in somatic cells from R6/2 transgenic Huntington's disease mice

Petersén, Åsa LU ; Stewénius, Ylva LU ; Björkqvist, Maria LU and Gisselsson, D (2005) In BMC Cell Biology 6(34).
Abstract
Background: Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by a CAG repeat expansion in the HD gene. The huntingtin protein expressed from HD has an unknown function but is suggested to interact with proteins involved in the cell division machinery. The R6/2 transgenic mouse is the most widely used model to study HD. In R6/2 fibroblast cultures, a reduced mitotic index and high frequencies of multiple centrosomes and aneuploid cells have recently been reported. Aneuploidy is normally a feature closely connected to neoplastic disease. To further explore this unexpected aspect of HD, we studied cultures derived from 6- and 12-week- old R6/2 fibroblasts, skeletal muscle cells, and liver cells. Results: Cytogenetic... (More)
Background: Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by a CAG repeat expansion in the HD gene. The huntingtin protein expressed from HD has an unknown function but is suggested to interact with proteins involved in the cell division machinery. The R6/2 transgenic mouse is the most widely used model to study HD. In R6/2 fibroblast cultures, a reduced mitotic index and high frequencies of multiple centrosomes and aneuploid cells have recently been reported. Aneuploidy is normally a feature closely connected to neoplastic disease. To further explore this unexpected aspect of HD, we studied cultures derived from 6- and 12-week- old R6/2 fibroblasts, skeletal muscle cells, and liver cells. Results: Cytogenetic analyses revealed a high frequency of polyploid cells in cultures from both R6/2 and wild-type mice with the greatest proportions of polyploid cells in cultures derived from skeletal muscle cells of both genotypes. The presence of polyploid cells in skeletal muscle in vivo was confirmed by fluorescence in situ hybridisation with centromeric probes. Enlarged and supernumerary centrosomes were found in cultures from both R6/ 2 and wild-type mice. However, no aneuploid cells could be found in any of the tissues. Conclusion: We conclude that polyploid cells are found in fibroblast and skeletal muscle cultures derived from R6/2 and wild-type littermate mice and that aneuploidy is unlikely to be a hallmark of HD. (Less)
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organization
publishing date
type
Contribution to journal
publication status
published
subject
in
BMC Cell Biology
volume
6
issue
34
publisher
BioMed Central
external identifiers
  • pmid:16159402
  • wos:000232164700001
  • scopus:25444469199
ISSN
1471-2121
DOI
10.1186/1471-2121-6-34
language
English
LU publication?
yes
id
53834d1e-106d-4c6e-81a5-db5bd260360e (old id 223486)
date added to LUP
2007-08-10 16:27:39
date last changed
2017-01-01 07:10:39
@article{53834d1e-106d-4c6e-81a5-db5bd260360e,
  abstract     = {Background: Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by a CAG repeat expansion in the HD gene. The huntingtin protein expressed from HD has an unknown function but is suggested to interact with proteins involved in the cell division machinery. The R6/2 transgenic mouse is the most widely used model to study HD. In R6/2 fibroblast cultures, a reduced mitotic index and high frequencies of multiple centrosomes and aneuploid cells have recently been reported. Aneuploidy is normally a feature closely connected to neoplastic disease. To further explore this unexpected aspect of HD, we studied cultures derived from 6- and 12-week- old R6/2 fibroblasts, skeletal muscle cells, and liver cells. Results: Cytogenetic analyses revealed a high frequency of polyploid cells in cultures from both R6/2 and wild-type mice with the greatest proportions of polyploid cells in cultures derived from skeletal muscle cells of both genotypes. The presence of polyploid cells in skeletal muscle in vivo was confirmed by fluorescence in situ hybridisation with centromeric probes. Enlarged and supernumerary centrosomes were found in cultures from both R6/ 2 and wild-type mice. However, no aneuploid cells could be found in any of the tissues. Conclusion: We conclude that polyploid cells are found in fibroblast and skeletal muscle cultures derived from R6/2 and wild-type littermate mice and that aneuploidy is unlikely to be a hallmark of HD.},
  author       = {Petersén, Åsa and Stewénius, Ylva and Björkqvist, Maria and Gisselsson, D},
  issn         = {1471-2121},
  language     = {eng},
  number       = {34},
  publisher    = {BioMed Central},
  series       = {BMC Cell Biology},
  title        = {Euploidy in somatic cells from R6/2 transgenic Huntington's disease mice},
  url          = {http://dx.doi.org/10.1186/1471-2121-6-34},
  volume       = {6},
  year         = {2005},
}