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Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease.

Petersén, Åsa LU ; Chase, K ; Puschban, Zoe LU ; DiFiglia, M ; Brundin, Patrik LU and Aronin, N (2002) In Experimental Neurology 175(1). p.297-300
Abstract
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... (More)
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. (Less)
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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
in
Experimental Neurology
volume
175
issue
1
pages
297 - 300
publisher
Elsevier
external identifiers
  • pmid:12009780
  • wos:000175532800026
  • scopus:0036254430
ISSN
0014-4886
DOI
10.1006/exnr.2002.7885
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
42d84e78-bc00-4337-ac17-442546151a75 (old id 108228)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12009780&dopt=Abstract
date added to LUP
2016-04-01 12:21:19
date last changed
2021-01-06 06:29:18
@article{42d84e78-bc00-4337-ac17-442546151a75,
  abstract     = {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.},
  author       = {Petersén, Åsa and Chase, K and Puschban, Zoe and DiFiglia, M and Brundin, Patrik and Aronin, N},
  issn         = {0014-4886},
  language     = {eng},
  number       = {1},
  pages        = {297--300},
  publisher    = {Elsevier},
  series       = {Experimental Neurology},
  title        = {Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease.},
  url          = {http://dx.doi.org/10.1006/exnr.2002.7885},
  doi          = {10.1006/exnr.2002.7885},
  volume       = {175},
  year         = {2002},
}