Prog Clin Biol Res. 1993;384:117-33.

Segmental trisomy as a mouse model for Down syndrome.

Davisson MT, Schmidt C, Reeves RH, Irving NG, Akeson EC, Harris BS, Bronson RT.

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Jackson Laboratory, Bar Harbor, Maine 04609.

Abstract

Mice trisomic for Chromosome (Chr) 16 have been used extensively as an animal model for human Down Syndrome (Trisomy 21). This system has drawbacks, however: trisomy for all of Chr 16 is incompatible with postnatal survival and produces trisomy for many more genes than those conserved in human Chr 21. We report here the development and preliminary characterization of mice that are trisomic for only the segment of mouse Chr 16 that is conserved in human Chr 21. While these segmentally trisomic mice, Ts(17(16)) 65Dn, do not appear to have all the features characteristic of Down Syndrome, they represent a mouse model that survives to adulthood and may be useful to study features of Down Syndrome that develop later in life, such as susceptibility to infection, increased incidence of leukemia, and Alzheimer-like neuropathology.

PMID:: 8115398; [PubMed - indexed for MEDLINE]

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Cited by 27 PubMed Central articles

Effects of Neonatal Neural Progenitor Cell Implantation on Adult Neuroanatomy and Cognition in the Ts65Dn Model of Down Syndrome. [PLoS One. 2012]
Effects of Neonatal Neural Progenitor Cell Implantation on Adult Neuroanatomy and Cognition in the Ts65Dn Model of Down Syndrome.
Rachubinski AL, Crowley SK, Sladek JR Jr, Maclean KN, Bjugstad KB. PLoS One. 2012; 7(4):e36082. Epub 2012 Apr 25.
Identification of the translocation breakpoints in the Ts65Dn and Ts1Cje mouse lines: relevance for modeling Down syndrome. [Mamm Genome. 2011]
Identification of the translocation breakpoints in the Ts65Dn and Ts1Cje mouse lines: relevance for modeling Down syndrome.
Duchon A, Raveau M, Chevalier C, Nalesso V, Sharp AJ, Herault Y. Mamm Genome. 2011 Dec; 22(11-12):674-84. Epub 2011 Sep 28.
The Down syndrome critical region regulates retinogeniculate refinement. [J Neurosci. 2011]
The Down syndrome critical region regulates retinogeniculate refinement.
Blank M, Fuerst PG, Stevens B, Nouri N, Kirkby L, Warrier D, Barres BA, Feller MB, Huberman AD, Burgess RW, et al. J Neurosci. 2011 Apr 13; 31(15):5764-76.

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Segmental trisomy as a mouse model for Down syndrome.

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