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Neuron
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Neuron
Article . 2008
License: Elsevier Non-Commercial
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Neuron
Article . 2008 . Peer-reviewed
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Suppression of Neurodegeneration and Increased Neurotransmission Caused by Expanded Full-Length Huntingtin Accumulating in the Cytoplasm

Authors: Cindy V. Ly; Patrik Verstreken; Robert E. Hughes; Eliana Romero; Juan Botas; Hugo J. Bellen; Guang Ho Cha;

Suppression of Neurodegeneration and Increased Neurotransmission Caused by Expanded Full-Length Huntingtin Accumulating in the Cytoplasm

Abstract

Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of a translated CAG repeat in the N terminus of the huntingtin (htt) protein. Here we describe the generation and characterization of a full-length HD Drosophila model to reveal a previously unknown disease mechanism that occurs early in the course of pathogenesis, before expanded htt is imported into the nucleus in detectable amounts. We find that expanded full-length htt (128Qhtt(FL)) leads to behavioral, neurodegenerative, and electrophysiological phenotypes. These phenotypes are caused by a Ca2+-dependent increase in neurotransmitter release efficiency in 128Qhtt(FL) animals. Partial loss of function in synaptic transmission (syntaxin, Snap, Rop) and voltage-gated Ca2+ channel genes suppresses both the electrophysiological and the neurodegenerative phenotypes. Thus, our data indicate that increased neurotransmission is at the root of neuronal degeneration caused by expanded full-length htt during early stages of pathogenesis.

Keywords

Cytoplasm, Neuroscience(all), Green Fluorescent Proteins, HUMDISEASE, Nerve Tissue Proteins, Eye, Synaptic Transmission, MOLNEURO, Animals, Genetically Modified, Animals, Humans, Huntingtin Protein, Neurotransmitter Agents, Behavior, Animal, Nuclear Proteins, Disease Models, Animal, Huntington Disease, Larva, Mutation, Nerve Degeneration, Microscopy, Electron, Scanning, Calcium, Drosophila

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    149
    popularity
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    Top 10%
    influence
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    impulse
    This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
    Top 1%
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selected citations
These citations are derived from selected sources.
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!
149
Top 10%
Top 10%
Top 1%
hybrid