Abstract

Polyglutamine (polyQ) disease is a group of inherited neurodegenerative disorders caused by abnormal elongation of an existing polyQ domain in disease proteins. Proteins that harbor the expanded polyQ domain cause progressive dysfunction and death of neurons. The nucleocytoplasmic transport of disease proteins displays a close correlation with polyQ toxicity. Classical nuclear localization signals (NLSs) and nuclear export signals (NESs) have been reported on different polyQ disease proteins and they play roles in regulating the subcellular localization and functions of disease proteins. In addition to classical NLSs and NESs, our preliminary data demonstrated that naked expanded polyQ domain also possesses nuclear transport signal activities. Elucidating the transport pathways that govern the nuclear translocation of polyQ proteins would provide extra insights into the pathogenesis of polyQ diseases.

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