Article
IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux.
Human molecular genetics - 1 Jan 2012
Lee Huikyong, Noh Jee-Yeon, Oh Yumin, Kim Youngdoo, Chang Jae-Woong, Chung Chul-Woong, Lee Soon-Tae, Kim Manho, Ryu Hoon, Jung Yong-Keun
Abstract excerpt
Huntington's disease (HD), an inherited neurodegenerative disorder, is caused by an expansion of cytosine-adenine-guanine repeats in the huntingtin gene. The aggregation of mutant huntingtin (mtHTT) and striatal cell loss are representative features to cause uncontrolled movement and cognitive defect in HD. However, underlying mechanism of mtHTT aggregation and cell toxicity remains still elusive. Here, to find...
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