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The Journal of Cell Biology
Article
License: CC BY NC SA
Data sources: UnpayWall
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PubMed Central
Other literature type . 2008
Data sources: PubMed Central
The Journal of Cell Biology
Article . 2008 . Peer-reviewed
Data sources: Crossref
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Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin

Authors: Wang, Chuan-En; Zhou, Hui; McGuire, John R.; Cerullo, Vincenzo; Lee, Brendan; Li, Shi-Hua; Li, Xiao-Jiang;

Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin

Abstract

Mutant huntingtin accumulates in the neuronal nuclei and processes, which suggests that its subcellular localization is critical for the pathology of Huntington's disease (HD). However, the contribution of cytoplasmic mutant huntingtin and its aggregates in neuronal processes (neuropil aggregates) has not been rigorously explored. We generated an intracellular antibody (intrabody) whose binding to a unique epitope of human huntingtin is enhanced by polyglutamine expansion. This intrabody decreases the cytotoxicity of mutant huntingtin and its distribution in neuronal processes. When expressed in the striatum of HD mice via adenoviral infection, the intrabody reduces neuropil aggregate formation and ameliorates neurological symptoms. Interaction of the intrabody with mutant huntingtin increases the ubiquitination of cytoplasmic huntingtin and its degradation. These findings suggest that the intrabody reduces the specific neurotoxicity of cytoplasmic mutant huntingtin and its associated neurological symptoms by preventing the accumulation of mutant huntingtin in neuronal processes and promoting its clearance in the cytoplasm.

Keywords

Cytoplasm, Huntingtin Protein, Neuropil, Sequence Homology, Amino Acid, Molecular Sequence Data, Antibodies, Monoclonal, Nuclear Proteins, Nerve Tissue Proteins, Models, Biological, Adenoviridae, Epitopes, Mice, Animals, Humans, Amino Acid Sequence, Nervous System Diseases, Research Articles

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    influence
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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
110
Top 10%
Top 10%
Top 1%
Green
hybrid