Ontology highlight
ABSTRACT:
SUBMITTER: Romero E
PROVIDER: S-EPMC2277511 | biostudies-literature | 2008 Jan
REPOSITORIES: biostudies-literature
Romero Eliana E Cha Guang-Ho GH Verstreken Patrik P Ly Cindy V CV Hughes Robert E RE Bellen Hugo J HJ Botas Juan J
Neuron 20080101 1
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 be ...[more]