Cystamine and intrabody co-treatment confers additional benefits in a fly model of Huntington's disease
Language: 
English
Abstract: 

Huntington's disease (HD) is a lethal, neurodegenerative disorder caused by expansion of the polyglutamine repeat in the Huntingtin gene (HTT), leading to mutant protein misfolding, aggregation, and neuronal death. Feeding a Drosophila HD model cystamine, or expressing a transgene encoding the anti-htt intracellular antibody (intrabody) C4-scFv in the nervous system, demonstrated therapeutic potential, but suppression of pathology was incomplete. We hypothesized that a combinatorial approach entailing drug and intrabody administration could enhance rescue of HD pathology in flies and that timing of treatment would affect outcomes. Feeding cystamine to adult HD flies expressing the intrabody resulted in a significant, additional rescue of photoreceptor neurodegeneration, but no additional benefit in longevity. Feeding cystamine during both larval and adult stages produced the converse result: longevity was significantly improved, but increased photoreceptor survival was not. We conclude that cystamine-intrabody combination therapies can be effective, reducing neurodegeneration and prolonging survival, depending on administration protocols.

Author(s): 
Bortvedt, S. F.
McLear, J. A.
Messer, A.
Ahern-Rindell, A. J.
Wolfgang, W. J.
Item Type: 
Journal Article
Publication Title: 
Neurobiology of Disease
Journal Abbreviation: 
Neurobiol. Dis.
Publication Date: 
2010-10
Publication Year: 
2010
Pages: 
130-134
Volume: 
40
Issue: 
1
ISSN: 
1095-953X
DOI: 
10.1016/j.nbd.2010.04.007
Library Catalog: 
NCBI Published Medical (?)
Extra: 
PMID: 20399860 PMCID: PMC2924926

Turabian/Chicago Citation

S. F. Bortvedt, J. A. McLear, A. Messer, A. J. Ahern-Rindell and W. J. Wolfgang. 2010-10. "Cystamine and intrabody co-treatment confers additional benefits in a fly model of Huntington's disease." Neurobiology of Disease 40: 1: 130-134. 10.1016/j.nbd.2010.04.007.

Wikipedia Citation

<ref> {{Cite journal | doi = 10.1016/j.nbd.2010.04.007 | issn = 1095-953X | volume = 40 | pages = 130-134 | last = Bortvedt | first = S. F. | coauthors = McLear, J. A., Messer, A., Ahern-Rindell, A. J., Wolfgang, W. J. | title = Cystamine and intrabody co-treatment confers additional benefits in a fly model of Huntington's disease | journal = Neurobiology of Disease | date = 2010-10 | pmid = | pmc = }} </ref>