Cited by 382Down syndrome and intellectual disability researchGenetics and Neurodevelopmental DisordersAlzheimer's disease research and treatments

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Ts1Cje, a partial trisomy 16 mouse model for Down syndrome, exhibits learning and behavioral abnormalities

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Sago, Haruhiko, Carlson, Elaine J., Smith, Desmond J., Kilbridge, Joshua, Rubin, Edward M., Mobley, William C., Epstein, Charles J., Huang, Ting-Ting·1998·Proceedings of the National Academy of Sciences

DOI 10.1073/pnas.95.11.6256PMC PMC27649

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Abstract

A mouse model for Down syndrome, Ts1Cje, has been developed. This model has made possible a step in the genetic dissection of the learning, behavioral, and neurological abnormalities associated with segmental trisomy for the region of mouse chromosome 16 homologous with the so-called “Down syndrome region” of human chromosome segment 21q22. Tests of learning in the Morris water maze and assessment of spontaneous locomotor activity reveal distinct learning and behavioral abnormalities, some of which are indicative of hippocampal dysfunction. The triplicated region in Ts1Cje, from Sod1 to Mx1 , is smaller than that in Ts65Dn, another segmental trisomy 16 mouse, and the learning deficits in Ts1Cje are less severe than those in Ts65Dn. In addition, degeneration of basal forebrain cholinergic neurons, which was observed in Ts65Dn, was absent in Ts1Cje.

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Authors: Sago, H., Carlson, E. J., Smith, D. J., Kilbridge, J., Rubin, E. M., Mobley, W. C., Epstein, C. J., & Huang, T.-T.
Affiliations: Lawrence Berkeley National Laboratory (US)
Corresponding author: Huang, Ting-Ting
ORCID: Sago, Haruhiko
Kilbridge, Joshua
Rubin, Edward M.
Mobley, William C.
Epstein, Charles J.
Huang, Ting-Ting
Venue: Proceedings of the National Academy of Sciences
Publisher: National Academy of Sciences
Published: 1998-05-26
Volume: 95
Issue: 11
Pages: 6256-6261
ISSN: 0027-8424 (ISSN-L)
1091-6490
DOI: 10.1073/pnas.95.11.6256
PMC: PMC27649
OpenAlex: W2079155607
Cited by: 382
References: 32
Citations by year: 2026: 3 · 2025: 5 · 2024: 8 · 2023: 7 · 2022: 10 · 2021: 14 · 2020: 9 · 2019: 12 · 2018: 11 · 2017: 20 · 2016: 13 · 2015: 8 · 2014: 14 · 2013: 14 · 2012: 23
FWCI: 19.96
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Locations: Proceedings of the National Academy of Sciences (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health (5p01hl018574-26, 5r01hd031498-05)
National Institute on Aging (AG-08938, P01AG008938)
National Heart, Lung, and Blood Institute (HL-18574)
Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD-31498)
Topics: Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Keywords: Down syndrome, mouse model, learning deficits, Morris water maze, locomotor activity, hippocampal dysfunction, basal forebrain cholinergic neurons, SOD1, MX1, chromosome 21q22
MeSH terms: Animals, Behavior, Animal, Disease Models, Animal, Down Syndrome, Humans, Learning Disabilities, Trisomy, Mice

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Ts1Cje, a partial trisomy 16 mouse model for Down syndrome, exhibits learning and behavioral abnormalities

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