Genetic deletion of transglutaminase 2 does not rescue the phenotypic deficits observed in R6/2 and zQ175 mouse models of Huntington's disease.

Huntington's disease (HD) is an autosomal dominant, progressive neurodegenerative disorder caused by expansion of CAG repeats in the huntingtin gene. Tissue transglutaminase 2 (TG2), a multi-functional enzyme, was found to be increased both in HD patients and in mouse models of the disease. Fur...

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Main Authors: Liliana B Menalled, Andrea E Kudwa, Steve Oakeshott, Andrew Farrar, Neil Paterson, Igor Filippov, Sam Miller, Mei Kwan, Michael Olsen, Jose Beltran, Justin Torello, Jon Fitzpatrick, Richard Mushlin, Kimberly Cox, Kristi McConnell, Matthew Mazzella, Dansha He, Georgina F Osborne, Rand Al-Nackkash, Gill P Bates, Pasi Tuunanen, Kimmo Lehtimaki, Dani Brunner, Afshin Ghavami, Sylvie Ramboz, Larry Park, Douglas Macdonald, Ignacio Munoz-Sanjuan, David Howland
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC4067284?pdf=render