Article
Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm.
Neuron - 10 Jan 2008
Romero Eliana, Cha Guang-Ho, Verstreken Patrik, Ly Cindy V, Hughes Robert E, Bellen Hugo J, Botas Juan
Abstract excerpt
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...
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