| Biography | |
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 Prof. Shihua Li Department of Human Genetics School of medicine, Emory University, China |
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| Title: Glial Function in polyglutamine expansion-mediated neurodegeneration | |
| Abstract:
Polyglutamine (PolyQ) expansion causes at least 9 neurodegenerative diseases. Including huntington’s disease (HD), Spinal cerebellum atxia (SCA) 1,2,3,6,8,17, spinal and bulbar muscular atrophy (SBMA), and Dentatorubral-pallidoluysian atrophy (DRPLA). In these diseases, mutant polyQ protein tends to misfold and forms aggregates in specific brain regions, leading to preferential neuronal loss in the selective brain regions. Many studies have focused on the neuronal loss in the brains of polyQ diseases mouse models and human patients. However, glial cells are the majority cell types in the brain, and whether mutant polyQ proteins cause functional defects in glia and whether such defects contribute to neurodegeneration in polyQ diseases remain to be fully investigated. We have studied the effect of polyQ expansion in the glial cells in HD and spinal cerebellum ataxia 17 (SCA-17). We found that the presence of mutant polyQ proteins in glial cells caused a number of glial dysfunctions, including BDNF release impairment, reduced mutant protein clearance and demyelination, which significantly contributed to the pathogenesis of the polyQ diseases. We also reported that mutant polyQ protein induces neurotoxicity via both cell autonomous and non-autonomous mechanisms.
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