Inactivation of Drosophila Huntingtin affects long-term adult functioning and the pathogenesis of a Huntington’s disease model

摘要: SUMMARY A polyglutamine expansion in the huntingtin ( HTT ) gene causes neurodegeneration Huntington’s disease (HD), but vivo function of native protein (Htt) is largely unknown. Numerous biochemical and vitro studies have suggested a role for Htt neuronal development, synaptic axonal trafficking. To test these models, we generated null mutant putative Drosophila homolog htt , hereafter referred to as dhtt and, surprisingly, found that animals are viable with no obvious developmental defects. Instead, required maintaining mobility long-term survival adult animals, modulating terminal complexity brain. Furthermore, removing endogenous significantly accelerates neurodegenerative phenotype associated model toxicity (HD-Q93), providing evidence disrupting normal might contribute HD pathogenesis.