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The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies.

Li, Jia-Yi LU ; Popovic, Natalija LU and Brundin, Patrik LU (2005) In NeuroRx2004-01-01+01:002007-01-01+01:00 2(3). p.64-447
Abstract
Huntington's disease (HD) is a genetic neurodegenerative disorder. Since identification of the disease-causing gene in 1993, a number of genetically modified animal models of HD have been generated. The first transgenic mouse models, R6/1 and R6/2 lines, were established 8 years ago. The R6/2 mice have been the best characterized and the most widely used model to study pathogenesis of HD and therapeutic interventions. In the present review, we especially focus on the characteristics of R6 transgenic mouse models and, in greater detail, describe the different therapeutic strategies that have been tested in these mice. We also, at the end, critically assess the relevance of the HD mouse models compared with the human disease and discuss how... (More)
Huntington's disease (HD) is a genetic neurodegenerative disorder. Since identification of the disease-causing gene in 1993, a number of genetically modified animal models of HD have been generated. The first transgenic mouse models, R6/1 and R6/2 lines, were established 8 years ago. The R6/2 mice have been the best characterized and the most widely used model to study pathogenesis of HD and therapeutic interventions. In the present review, we especially focus on the characteristics of R6 transgenic mouse models and, in greater detail, describe the different therapeutic strategies that have been tested in these mice. We also, at the end, critically assess the relevance of the HD mouse models compared with the human disease and discuss how they can be best used in the future. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Huntington Disease: psychology, Huntington Disease: therapy, Mice, Huntington Disease: pathology, Behavior, Animal, Humans, Huntington Disease: diet therapy, Huntington Disease: genetics, Animals, Anti-Inflammatory Agents: therapeutic use, Apoptosis: drug effects, Brain: pathology, Disease Models, Transgenic: physiology, Protease Inhibitors: therapeutic use, Protein Folding, Research Support, Non-U.S. Gov't, Tissue Therapy, Transglutaminases: antagonists & inhibitors
in
NeuroRx2004-01-01+01:002007-01-01+01:00
volume
2
issue
3
pages
64 - 447
publisher
Springer
external identifiers
  • scopus:26844498655
ISSN
1545-5343
DOI
10.1602/neurorx.2.3.447
language
English
LU publication?
yes
id
a302e2d4-d61d-4a36-b52d-df78cf5011d9 (old id 150583)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16389308&dopt=Abstract
date added to LUP
2007-07-16 15:00:32
date last changed
2017-11-12 03:56:37
@article{a302e2d4-d61d-4a36-b52d-df78cf5011d9,
  abstract     = {Huntington's disease (HD) is a genetic neurodegenerative disorder. Since identification of the disease-causing gene in 1993, a number of genetically modified animal models of HD have been generated. The first transgenic mouse models, R6/1 and R6/2 lines, were established 8 years ago. The R6/2 mice have been the best characterized and the most widely used model to study pathogenesis of HD and therapeutic interventions. In the present review, we especially focus on the characteristics of R6 transgenic mouse models and, in greater detail, describe the different therapeutic strategies that have been tested in these mice. We also, at the end, critically assess the relevance of the HD mouse models compared with the human disease and discuss how they can be best used in the future.},
  author       = {Li, Jia-Yi and Popovic, Natalija and Brundin, Patrik},
  issn         = {1545-5343},
  keyword      = {Huntington Disease: psychology,Huntington Disease: therapy,Mice,Huntington Disease: pathology,Behavior,Animal,Humans,Huntington Disease: diet therapy,Huntington Disease: genetics,Animals,Anti-Inflammatory Agents: therapeutic use,Apoptosis: drug effects,Brain: pathology,Disease Models,Transgenic: physiology,Protease Inhibitors: therapeutic use,Protein Folding,Research Support,Non-U.S. Gov't,Tissue Therapy,Transglutaminases: antagonists & inhibitors},
  language     = {eng},
  number       = {3},
  pages        = {64--447},
  publisher    = {Springer},
  series       = {NeuroRx2004-01-01+01:002007-01-01+01:00},
  title        = {The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies.},
  url          = {http://dx.doi.org/10.1602/neurorx.2.3.447},
  volume       = {2},
  year         = {2005},
}