Article
Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo.
American journal of human genetics - 1 Feb 2001
Leavitt B R, Guttman J A, Hodgson J G, Kimel G H, Singaraja R, Vogl A W, Hayden M R
Abstract excerpt
We have developed yeast artificial chromosome (YAC) transgenic mice expressing normal (YAC18) and mutant (YAC46 or YAC72) human huntingtin (htt), in a developmental- and tissue-specific manner, that is identical to endogenous htt. YAC72 mice develop selective degeneration of medium spiny projection neurons in the lateral striatum, similar to what is observed in Huntington disease. Mutant human htt expressed by...
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