Journal
MOLECULAR BRAIN RESEARCH
Volume 121, Issue 1-2, Pages 141-145Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.molbrainres.2003.11.011
Keywords
Huntington's disease; intrabody; malonate; organotypic slice culture; polyglutamine; biolistics; sFv
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Funding
- NINDS NIH HHS [NS39946, NS38002] Funding Source: Medline
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Huntington's disease (HD) is a progressive, hereditary, neurodegenerative disorder caused by an expanded polyglutamine tract in huntingtin protein, leading to misfolding and abnormal protein-protein interactions. Reducing the initial misfolding should lead to decreased pathogenesis. We show that malonate stress increases the number of dead or dying cells when organotypic slice cultures are transduced to express pathological-length huntingtin fragments. Co-transfected anti-HD single-chain Fv (sFv) intrabodies can reverse this HD-specific increase in malonate-induced morbidity. (C) 2003 Elsevier B.V. All rights reserved.
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