Article
Peripheral huntingtin silencing does not ameliorate central signs of disease in the B6.HttQ111/+ mouse model of Huntington's disease.
PloS one - 1 Jan 2017
Coffey Sydney R, Bragg Robert M, Minnig Shawn, Ament Seth A, Cantle Jeffrey P, Glickenhaus Anne, Shelnut Daniel, Carrillo José M, Shuttleworth Dominic D, Rodier Julie-Anne, Noguchi Kimihiro, Bennett C Frank, Price Nathan D, Kordasiewicz Holly B, Carroll Jeffrey B
Abstract excerpt
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease whose predominant neuropathological signature is the selective loss of medium spiny neurons in the striatum. Despite this selective neuropathology, the mutant protein (huntingtin) is found in virtually every cell so far studied, and, consequently, phenotypes are observed in a wide range of organ systems both inside and outside the...
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