Title : Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity.

Pub. Date : 2008 Dec 1

PMID : 19015277






3 Functional Relationships(s)
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1 We further decipher the mechanistic basis for this defect in ERAD: the entrapment of the essential ERAD proteins Npl4, Ufd1, and p97 by polyQ-expanded htt fragments. polyglutamine huntingtin Rattus norvegicus
2 Our results establish that impaired ER protein homeostasis is a broad and highly conserved contributor to polyQ toxicity in yeast, in PC12 cells, and, importantly, in striatal cells expressing full-length polyQ-expanded huntingtin. polyglutamine huntingtin Rattus norvegicus
3 Our results establish that impaired ER protein homeostasis is a broad and highly conserved contributor to polyQ toxicity in yeast, in PC12 cells, and, importantly, in striatal cells expressing full-length polyQ-expanded huntingtin. polyglutamine huntingtin Rattus norvegicus