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Huntington's disease - The sting in the tail

Research output: Contribution to journalComment/debate

Maria Jimenez-Sanchez, David C. Rubinsztein

Original languageEnglish
Pages (from-to)2215-2216
Number of pages2
JournalThe EMBO journal
Volume34
Issue number17
Early online date29 Jul 2015
DOIs
E-pub ahead of print29 Jul 2015
Published2 Sep 2015

King's Authors

Abstract

Huntington's disease (HD) is a progressive neurodegenerative condition caused by the abnormal expansion of a polyglutamine tract in the N-terminus of the huntingtin protein. Over the last 20 years, HD pathogenesis has been explained by the generation of N-terminal fragments containing the polyglutamine stretch. A new study from Frederic Saudou's group now investigates the function of the C-terminal fragments generated upon cleavage and shows that these products may also contribute to cellular toxicity in HD (El-Daher et al,). While it is well established that abnormal expansion of a polyQ tract in the huntingtin protein N-terminus triggers Huntington's disease, recent findings show that C-terminal cleavage fragments also contribute to cellular toxicity.

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