Developmental abnormalities and age-related neurodegeneration in a mouse model of Down syndrome
Open Access
- 12 November 1996
- journal article
- research article
- Published by Proceedings of the National Academy of Sciences in Proceedings of the National Academy of Sciences
- Vol. 93 (23) , 13333-13338
- https://doi.org/10.1073/pnas.93.23.13333
Abstract
To study the pathogenesis of central nervous system abnormalities in Down syndrome (DS), we have analyzed a new genetic model of DS, the partial trisomy 16 (Ts65Dn) mouse. Ts65Dn mice have an extra copy of the distal aspect of mouse chromosome 16, a segment homologous to human chromosome 21 that contains much of the genetic material responsible for the DS phenotype. Ts65Dn mice show developmental delay during the postnatal period as well as abnormal behaviors in both young and adult animals that may be analogous to mental retardation. Though the Ts65Dn brain is normal on gross examination, there is age-related degeneration of septohippocampal cholinergic neurons and astrocytic hypertrophy, markers of the Alzheimer disease pathology that is present in elderly DS individuals. These findings suggest that Ts65Dn mice may be used to study certain developmental and degenerative abnormalities in the DS brain.Keywords
This publication has 47 references indexed in Scilit:
- A behavioral assessment of Ts65Dn mice: a putative Down syndrome modelNeuroscience Letters, 1995
- Mental modellingNature Genetics, 1995
- ApoE genotype and Down's syndromeThe Lancet, 1994
- Introduction and expression of the 400 kilobase precursor amyloid protein gene in transgenic miceNature Genetics, 1993
- Neonatal exposure to bFGF exerts NGF-like effects on mouse behavioral developmentNeurotoxicology and Teratology, 1993
- Evolutionary Conservation of the Mouse Apolipoprotein e-c1-c2 Gene Cluster: Structure and Genetic Variability in Inbred MiceGenomics, 1993
- Amyloid A4 Protein and Its Precursor in Down's Syndrome and Alzheimer's DiseaseNew England Journal of Medicine, 1989
- Expression of the Alzheimer amyloid precursor gene transcripts in the human brainNeuron, 1988
- Down syndrome, Alzheimer's disease and the trisomy 16 mouseTrends in Neurosciences, 1988
- Astrocytes synthesize apolipoprotein E and metabolize apolipoprotein E-containing lipoproteinsBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1987