Article
Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment.
Human molecular genetics - 15 Apr 2011
Tebbenkamp Andrew T N, Swing Debbie, Tessarollo Lino, Borchelt David R
Abstract excerpt
Huntington's disease (HD) is a fatal neurodegenerative disease characterized pathologically by aggregates composed of N-terminal fragments of the mutant form of the protein huntingtin (htt). The role of these N-terminal fragments in disease pathogenesis has been questioned based in part on studies in transgenic mice. In one important example, mice that express an N-terminal fragment of mutant htt terminating at...
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