Article
Early autophagic response in a novel knock-in model of Huntington disease.
Human molecular genetics - 1 Oct 2010
Heng Mary Y, Duong Duy K, Albin Roger L, Tallaksen-Greene Sara J, Hunter Jesse M, Lesort Mathieu J, Osmand Alex, Paulson Henry L, Detloff Peter J
Abstract excerpt
The aggregation of mutant polyglutamine (polyQ) proteins has sparked interest in the role of protein quality-control pathways in Huntington's disease (HD) and related polyQ disorders. Employing a novel knock-in HD mouse model, we provide in vivo evidence of early, sustained alterations of autophagy in response to mutant huntingtin (mhtt). The HdhQ200 knock-in model, derived from the selective breeding of HdhQ150...
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