The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies.
(2005) In NeuroRx 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:
https://lup.lub.lu.se/record/150583
- author
- Li, Jia-Yi LU ; Popovic, Natalija LU and Brundin, Patrik LU
- organization
- publishing date
- 2005
- 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
- NeuroRx
- 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
- additional info
- The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Neuronal Survival (013212041)
- 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
- 2016-04-01 16:13:33
- date last changed
- 2022-04-15 02:59:48
@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}}, 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}}, language = {{eng}}, number = {{3}}, pages = {{64--447}}, publisher = {{Springer}}, series = {{NeuroRx}}, 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}}, doi = {{10.1602/neurorx.2.3.447}}, volume = {{2}}, year = {{2005}}, }