Proliferation deficits and gene expression dysregulation in Down's syndrome (Ts1Cje) neural progenitor cells cultured from neurospheres

Moldrich, Randal X., Dauphinot, Luce, Laffaire, Julien, Vitalis, Tania, Herault, Yann, Beart, Philip M., Rossier, Jean, Vivien, Denis, Gehrig, Corinne, Antonarakis, Stylianos E., Lyle, Robert and Potier, Marie-Claude (2009) Proliferation deficits and gene expression dysregulation in Down's syndrome (Ts1Cje) neural progenitor cells cultured from neurospheres. Journal of Neuroscience Research, 87 14: 3143-3152. doi:10.1002/jnr.22131


Author Moldrich, Randal X.
Dauphinot, Luce
Laffaire, Julien
Vitalis, Tania
Herault, Yann
Beart, Philip M.
Rossier, Jean
Vivien, Denis
Gehrig, Corinne
Antonarakis, Stylianos E.
Lyle, Robert
Potier, Marie-Claude
Title Proliferation deficits and gene expression dysregulation in Down's syndrome (Ts1Cje) neural progenitor cells cultured from neurospheres
Journal name Journal of Neuroscience Research   Check publisher's open access policy
ISSN 0360-4012
1097-4547
Publication date 2009-11
Year available 2009
Sub-type Article (original research)
DOI 10.1002/jnr.22131
Volume 87
Issue 14
Start page 3143
End page 3152
Total pages 10
Editor Jean de Vellis
Place of publication United States
Publisher John Wiley & Sons, Inc.
Collection year 2010
Language eng
Subject 730104 Nervous system and disorders
060405 Gene Expression (incl. Microarray and other genome-wide approaches)
110903 Central Nervous System
Abstract Down's syndrome neurophenotypes are characterized by mental retardation and a decreased brain volume. To identify whether deficits in proliferation could be responsible for this phenotype, neural progenitor cells were isolated from the developing E14 neocortex of Down's syndrome partial trisomy Ts1Cje mice and euploid (WT) littermates and grown as neurospheres. Ts1Cje neural progenitors proliferated at a slower rate, because of a longer cell cycle, and a greater number of cells were positive for glial fibrillary acidic protein. An increase in cell death was also noted. Gene expression profiles of neural progenitor cells from Ts1Cje and WT showed that 54% of triploid genes had expression ratios (Ts1Cje/WT) significantly greater than the expected diploid gene ratio of 1.0. Some diploid genes associated with proliferation, differentiation, and glial function were dysregulated. Interestingly, proliferation and gene expression dysregulation detected in the Ts1Cje mice did not require overexpression of the chromosome 21 genes amyloid precursor protein (App) and soluble superoxide dismutase 1 (Sod1).
Keyword Down's syndrome
Gene expression
Neocortex
Neural progenitor cells
Neurosphere
Proliferation
Ts1Cje
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: 2010 Higher Education Research Data Collection
Queensland Brain Institute Publications
 
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Created: Wed, 21 Oct 2009, 10:01:16 EST by Debra McMurtrie on behalf of Queensland Brain Institute