Rodent models in Down syndrome research: impact and future opportunities
Down syndrome is caused by trisomy of chromosome 21. To date, a multiplicity of mouse models with Down-syndrome-related features has been developed to understand this complex human chromosomal disorder. These mouse models have been important for determining genotype-phenotype relationships and ident...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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The Company of Biologists
2017-10-01
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| Series: | Disease Models & Mechanisms |
| Subjects: | |
| Online Access: | http://dmm.biologists.org/content/10/10/1165 |
| _version_ | 1818198391144841216 |
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| author | Yann Herault Jean M. Delabar Elizabeth M. C. Fisher Victor L. J. Tybulewicz Eugene Yu Veronique Brault |
| author_facet | Yann Herault Jean M. Delabar Elizabeth M. C. Fisher Victor L. J. Tybulewicz Eugene Yu Veronique Brault |
| author_sort | Yann Herault |
| collection | DOAJ |
| description | Down syndrome is caused by trisomy of chromosome 21. To date, a multiplicity of mouse models with Down-syndrome-related features has been developed to understand this complex human chromosomal disorder. These mouse models have been important for determining genotype-phenotype relationships and identification of dosage-sensitive genes involved in the pathophysiology of the condition, and in exploring the impact of the additional chromosome on the whole genome. Mouse models of Down syndrome have also been used to test therapeutic strategies. Here, we provide an overview of research in the last 15 years dedicated to the development and application of rodent models for Down syndrome. We also speculate on possible and probable future directions of research in this fast-moving field. As our understanding of the syndrome improves and genome engineering technologies evolve, it is necessary to coordinate efforts to make all Down syndrome models available to the community, to test therapeutics in models that replicate the whole trisomy and design new animal models to promote further discovery of potential therapeutic targets. |
| first_indexed | 2024-12-12T02:05:07Z |
| format | Article |
| id | doaj.art-790ab10207ce407489ca7bf4394b9b77 |
| institution | Directory Open Access Journal |
| issn | 1754-8403 1754-8411 |
| language | English |
| last_indexed | 2024-12-12T02:05:07Z |
| publishDate | 2017-10-01 |
| publisher | The Company of Biologists |
| record_format | Article |
| series | Disease Models & Mechanisms |
| spelling | doaj.art-790ab10207ce407489ca7bf4394b9b772022-12-22T00:42:05ZengThe Company of BiologistsDisease Models & Mechanisms1754-84031754-84112017-10-0110101165118610.1242/dmm.029728029728Rodent models in Down syndrome research: impact and future opportunitiesYann Herault0Jean M. Delabar1Elizabeth M. C. Fisher2Victor L. J. Tybulewicz3Eugene Yu4Veronique Brault5 Institut de Génétique et de Biologie Moléculaire et Cellulaire, Illkirch, 1 rue Laurent Fries, 67404 Illkirch, France T21 Research Society, Brain and Spine Institute (ICM), 75013 Paris T21 Research Society, Brain and Spine Institute (ICM), 75013 Paris T21 Research Society, Brain and Spine Institute (ICM), 75013 Paris T21 Research Society, Brain and Spine Institute (ICM), 75013 Paris Institut de Génétique et de Biologie Moléculaire et Cellulaire, Illkirch, 1 rue Laurent Fries, 67404 Illkirch, France Down syndrome is caused by trisomy of chromosome 21. To date, a multiplicity of mouse models with Down-syndrome-related features has been developed to understand this complex human chromosomal disorder. These mouse models have been important for determining genotype-phenotype relationships and identification of dosage-sensitive genes involved in the pathophysiology of the condition, and in exploring the impact of the additional chromosome on the whole genome. Mouse models of Down syndrome have also been used to test therapeutic strategies. Here, we provide an overview of research in the last 15 years dedicated to the development and application of rodent models for Down syndrome. We also speculate on possible and probable future directions of research in this fast-moving field. As our understanding of the syndrome improves and genome engineering technologies evolve, it is necessary to coordinate efforts to make all Down syndrome models available to the community, to test therapeutics in models that replicate the whole trisomy and design new animal models to promote further discovery of potential therapeutic targets.http://dmm.biologists.org/content/10/10/1165Down syndromeMouse modelChromosome engineeringAneuploidyDosage-senstive gene |
| spellingShingle | Yann Herault Jean M. Delabar Elizabeth M. C. Fisher Victor L. J. Tybulewicz Eugene Yu Veronique Brault Rodent models in Down syndrome research: impact and future opportunities Disease Models & Mechanisms Down syndrome Mouse model Chromosome engineering Aneuploidy Dosage-senstive gene |
| title | Rodent models in Down syndrome research: impact and future opportunities |
| title_full | Rodent models in Down syndrome research: impact and future opportunities |
| title_fullStr | Rodent models in Down syndrome research: impact and future opportunities |
| title_full_unstemmed | Rodent models in Down syndrome research: impact and future opportunities |
| title_short | Rodent models in Down syndrome research: impact and future opportunities |
| title_sort | rodent models in down syndrome research impact and future opportunities |
| topic | Down syndrome Mouse model Chromosome engineering Aneuploidy Dosage-senstive gene |
| url | http://dmm.biologists.org/content/10/10/1165 |
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