Depletion of Cognate Charged Transfer RNA Causes Translational Frameshifting within the Expanded CAG Stretch in Huntingtin

Huntington disease (HD), a dominantly inherited neurodegenerative disorder caused by the expansion of a CAG-encoded polyglutamine (polyQ) repeat in huntingtin (Htt), displays a highly heterogeneous etiopathology and disease onset. Here, we show that the translation of expanded CAG repeats in mutant...

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Bibliographic Details
Main Authors: Hannah Girstmair, Paul Saffert, Sascha Rode, Andreas Czech, Gudrun Holland, Norbert Bannert, Zoya Ignatova
Format: Article
Language:English
Published: Elsevier 2013-01-01
Series:Cell Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124713000065