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Why Huntington's proteins pile up: Two key tags guide their disposal

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There is no known cure for Huntington’s disease. A genetic mutation creates harmful proteins that accumulate and cause the disease’s typical symptoms. A team from the Department of Human Genetics at Ruhr University Bochum, Germany, has now shown how targeted ubiquitin tagging at two positions in the mutated huntingtin protein affects its breakdown and distribution within the cell. This insight could provide a starting point for future therapies. The team in Bochum, led by Professor Hoa Huu Phuc Nguyen, worked closely with Israeli researcher Professor Aaron Ciechanover, who was awarded the Nobel Prize in Chemistry in 2004 for his work on the protein degradation system.


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