Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Herault, Yann, Delabar, Jean M., Fisher, Elizabeth M. C., Tybulewicz, Victor L. J., Yu, Eugene, Brault, Veronique
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5665454/
https://www.ncbi.nlm.nih.gov/pubmed/28993310
http://dx.doi.org/10.1242/dmm.029728
_version_ 1783275149606256640
author Herault, Yann
Delabar, Jean M.
Fisher, Elizabeth M. C.
Tybulewicz, Victor L. J.
Yu, Eugene
Brault, Veronique
author_facet Herault, Yann
Delabar, Jean M.
Fisher, Elizabeth M. C.
Tybulewicz, Victor L. J.
Yu, Eugene
Brault, Veronique
author_sort Herault, Yann
collection PubMed
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.
format Online
Article
Text
id pubmed-5665454
institution National Center for Biotechnology Information
language English
publishDate 2017
publisher The Company of Biologists Ltd
record_format MEDLINE/PubMed
spelling pubmed-56654542017-11-09 Rodent models in Down syndrome research: impact and future opportunities Herault, Yann Delabar, Jean M. Fisher, Elizabeth M. C. Tybulewicz, Victor L. J. Yu, Eugene Brault, Veronique Dis Model Mech Review 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. The Company of Biologists Ltd 2017-10-01 /pmc/articles/PMC5665454/ /pubmed/28993310 http://dx.doi.org/10.1242/dmm.029728 Text en © 2017. Published by The Company of Biologists Ltd http://creativecommons.org/licenses/by/3.0This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.
spellingShingle Review
Herault, Yann
Delabar, Jean M.
Fisher, Elizabeth M. C.
Tybulewicz, Victor L. J.
Yu, Eugene
Brault, Veronique
Rodent models in Down syndrome research: impact and future opportunities
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 Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5665454/
https://www.ncbi.nlm.nih.gov/pubmed/28993310
http://dx.doi.org/10.1242/dmm.029728
work_keys_str_mv AT heraultyann rodentmodelsindownsyndromeresearchimpactandfutureopportunities
AT delabarjeanm rodentmodelsindownsyndromeresearchimpactandfutureopportunities
AT fisherelizabethmc rodentmodelsindownsyndromeresearchimpactandfutureopportunities
AT tybulewiczvictorlj rodentmodelsindownsyndromeresearchimpactandfutureopportunities
AT yueugene rodentmodelsindownsyndromeresearchimpactandfutureopportunities
AT braultveronique rodentmodelsindownsyndromeresearchimpactandfutureopportunities