WWW.JNEUROSCI.ORG
-
The Journal of Neuroscience
QUICK SEARCH:   [advanced]


     
-


HOME  |   SEARCH  |   ARCHIVE  |   SUBSCRIBE  |   CONTACT  |   HELP
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Submit an eLetter
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Web of Science (30)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Senut, M.-C.
Right arrow Articles by Gage, F. H.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Senut, M.-C.
Right arrow Articles by Gage, F. H.

 Previous Article  |  Next Article 

The Journal of Neuroscience, January 1, 2000, 20(1):219-229

Intraneuronal Aggregate Formation and Cell Death after Viral Expression of Expanded Polyglutamine Tracts in the Adult Rat Brain

Marie-Claude Senut, Steven T. Suhr, Brian Kaspar, and Fred H. Gage

Laboratory of Genetics, The Salk Institute for Biological Studies, La Jolla, California 92037

Expanded polyglutamine (polyQ) tracts have been linked to a new class of human disease characterized by psychiatric/motor syndromes associated with specific patterns of neurodegeneration. We have used a direct viral approach to locally express expanded polyglutamine tracts fused to the green fluorescent protein (97Q-GFP) in the adult rat brain. We show that intrastriatal expression of 97Q-GFP causes the rapid formation of fibrillar, cytoplasmic, and ubiquitinated nuclear aggregates in neurons. 97Q-GFP expression also results in a specific temporal pattern of cell death in the striatum. Co-infection studies suggest that high level 97Q-GFP-expressing cells die during the first month, whereas low level 97Q-GFP-expressing neurons persist for up to 6 months after infection. These data indicate that cumulative expression of polyQ repeats throughout the life of the animal is not required to induce neuronal death, but rather acute overexpression of polyQ is toxic to adult neurons in vivo.

Key words: polyglutamine repeats; gene transfer; adeno-associated viral vectors; brain; rat; Huntington's disease; aggregates

Copyright © 2000 Society for Neuroscience  0270-6474/0/201219-11$05.00/0


This article has been cited by other articles:


Home page
 StrokeHome page
G.K. Rajanikant, D. Zemke, M.-C. Senut, M. B. Frenkel, A. F. Chen, R. Gupta, and A. Majid
Carnosine Is Neuroprotective Against Permanent Focal Cerebral Ischemia in Mice
Stroke, November 1, 2007; 38(11): 3023 - 3031.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
L. P. de Almeida, C. A. Ross, D. Zala, P. Aebischer, and N. Deglon
Lentiviral-Mediated Delivery of Mutant Huntingtin in the Striatum of Rats Induces a Selective Neuropathology Modulated by Polyglutamine Repeat Size, Huntingtin Expression Levels, and Protein Length
J. Neurosci., May 1, 2002; 22(9): 3473 - 3483.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
S. T. Suhr, M.-C. Senut, J. P. Whitelegge, K. F. Faull, D. B. Cuizon, and F. H. Gage
Identities of Sequestered Proteins in Aggregates from Cells with Induced Polyglutamine Expression
J. Cell Biol., April 16, 2001; 153(2): 283 - 294.
[Abstract] [Full Text] [PDF]


-

Home  |   Search  |   Archive  |   Subscribe  |   Contact  |   Help
-
Copyright 2009 by Society for Neuroscience ONLINE ISSN: 1529-2401
-