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Cited by 458

The DOI matches its Crossref record; 30 of the 30 references it cites were found in a registry or an index.

Transgenic mice with increased Cu/Zn-superoxide dismutase activity: animal model of dosage effects in Down syndrome.

Open access
Epstein, C J, Avraham, K B, Lovett, M, Smith, S, Elroy-Stein, O, Rotman, G, Bry, C, Groner, Y·1987·Proceedings of the National Academy of Sciences

Abstract

Down syndrome, the phenotypic expression of human trisomy 21, is presumed to result from a 1.5-fold increase in the expression of the genes on human chromosome 21. As an approach to the development of an animal model for Down syndrome, several strains of transgenic mice that carry the human Cu/Zn-superoxide dismutase gene have been prepared. These animals express the transgene in a manner similar to that of humans, with 0.9- and 0.7-kilobase transcripts in a 1:4 ratio, and synthesize the human enzyme in an active form capable of forming human-mouse enzyme heterodimers. Cu/Zn-superoxide superoxide dismutase activity is increased from 1.6- to 6.0-fold in the brains of four transgenic strains and to an equal or lesser extent in several other tissues. These animals provide a unique system for studying the consequences of increased dosage of the Cu/Zn-superoxide dismutase gene in Down syndrome and the role of this enzyme in a variety of other pathological processes.

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Metadata

Authors
Epstein, C. J., Avraham, K. B., Lovett, M., Smith, S., Elroy-Stein, O., Rotman, G., Bry, C., & Groner, Y.
Venue
Proceedings of the National Academy of Sciences
Published
1987-11
Volume
84
Issue
22
Pages
8044-8048
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
OpenAlex
W2017199287
Cited by
458
References
30
FWCI
11.82
Type
journal article
Language
en
Open access
green

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The paper
Epstein, C. J., Avraham, K. B., Lovett, M., Smith, S., Elroy-Stein, O., Rotman, G., Bry, C., & Groner, Y. (1987). Transgenic mice with increased Cu/Zn-superoxide dismutase activity: animal model of dosage effects in Down syndrome. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.84.22.8044

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.