Article
Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease.
Human molecular genetics - 15 May 2011
Munsie Lise, Caron Nicholas, Atwal Randy Singh, Marsden Ian, Wild Edward J, Bamburg James R, Tabrizi Sarah J, Truant Ray
Abstract excerpt
Huntington's disease (HD) is caused by an expanded CAG tract in the Interesting transcript 15 (IT15) gene encoding the 350 kDa huntingtin protein. Cellular stresses can trigger the release of huntingtin from the endoplasmic reticulum, allowing huntingtin nuclear entry. Here, we show that endogenous, full-length huntingtin localizes to nuclear cofilin-actin rods during stress and is required for the proper stress...
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