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)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/223486
- author
- Petersén, Åsa
LU
; Stewénius, Ylva
LU
; Björkqvist, Maria
LU
and Gisselsson, D
- organization
- publishing date
- 2005
- type
- Contribution to journal
- publication status
- published
- subject
- in
- BMC Cell Biology
- volume
- 6
- issue
- 34
- publisher
- BioMed Central (BMC)
- 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
- 2016-04-01 16:38:10
- date last changed
- 2023-09-04 22:25:16
@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 (BMC)}},
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}},
doi = {{10.1186/1471-2121-6-34}},
volume = {{6}},
year = {{2005}},
}