Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease.
Author
Summary, in English
A transgenic mouse model of Huntington's disease (R6/1 and R6/2 lines) expressing exon 1 of the HD gene with 115-150 CAG repeats resisted striatal damage following injection of quinolinic acid and other neurotoxins. We examined whether excitotoxin resistance characterizes mice with mutant huntingtin transgenes. In a new transgenic mouse with 3 kb of mutant human huntingtin cDNA with 18, 46, or 100 CAG repeats, we found no change in susceptibility to intrastriatal injections of the excitotoxin quinolinic acid, compared to wild-type littermates. The new transgenic mice were injected with the same dose of quinolinic acid (30 nmol) as had been the R6 mice. Our findings highlight the importance of studying pathogenetic mechanisms in different transgenic models of a disease.
Department/s
Publishing year
2002
Language
English
Pages
297-300
Publication/Series
Experimental Neurology
Volume
175
Issue
1
Links
Document type
Journal article
Publisher
Elsevier
Topic
- Neurology
Keywords
- Huntington Disease : complications
- Heterozygote
- Huntington Disease : chemically induced
- Human
- Huntington Disease : genetics
- P.H.S.
- U.S. Gov't
- Support
- Non-P.H.S.
- Non-U.S. Gov't
- Species Specificity
- Quinolinic Acid : administration & dosage
- Neurons : pathology
- Neurons : drug effects
- Neurodegenerative Diseases : pathology
- Neurodegenerative Diseases : complications
- Neurodegenerative Diseases : chemically induced
- Microinjections
- Transgenic
- Mice
- Huntington Disease : pathology
- Disease Susceptibility
- Animal
- Disease Models
- Corpus Striatum : pathology
- Cell Count
- Cell Death
- Corpus Striatum : drug effects
Status
Published
ISBN/ISSN/Other
- ISSN: 0014-4886