Proliferation deficits and gene expression dysregulation in Down&#039;s syndrome (Ts1Cje) neural progenitor cells cultured from neurospheres

Moldrich, R. X.; Dauphinot, Luce; Laffaire, Julien; Vitalis, Tania; Herault, Yann; Beart, P. M.; Rossier, Jean; Vivien, Denis; Gehrig, Corinne; Antonarakis, Stylianos; Lyle, Robert; Potier, M. C.

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Title		Proliferation deficits and gene expression dysregulation in Down's syndrome (Ts1Cje) neural progenitor cells cultured from neurospheres
Authors		Moldrich, R. X. Dauphinot, Luce Laffaire, Julien Vitalis, Tania Herault, Yann Beart, P. M. Rossier, Jean Vivien, Denis Gehrig, Corinne Antonarakis, Stylianos Lyle, Robert Potier, M. C. show all authors [1 - 12]
Published in		Journal of Neuroscience Research. 2009, vol. 87, no. 14, p. 3143-3152
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).
Keywords		Animals — Cell Differentiation/genetics — Cell Proliferation — Cells, Cultured — Disease Models, Animal — Down Syndrome — Gene Expression/ physiology — Gene Expression Profiling — Genotype — Immunohistochemistry — Male — Mice — Microspheres — Neocortex/physiopathology — Neuroglia/pathology/physiology — Neurons/ pathology/ physiology — Oligonucleotide Array Sequence Analysis — Reverse Transcriptase Polymerase Chain Reaction — Stem Cells/ pathology/physiology
Identifiers		DOI: 10.1002/jnr.22131 PMID: 19472221
Full text		Article - Limited access to UNIGE Other version: http://www3.interscience.wiley.com/journal/122402972/abstract
Structures		Faculté de médecine / Section de médecine clinique / Département de médecine interne des spécialités
Citation (ISO format)		MOLDRICH, R. X. et al. Proliferation deficits and gene expression dysregulation in Down's syndrome (Ts1Cje) neural progenitor cells cultured from neurospheres. In: Journal of Neuroscience Research, 2009, vol. 87, n° 14, p. 3143-3152. https://archive-ouverte.unige.ch/unige:8907