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Article

Transgenic Mice in the Study of Polyglutamine Repeat Expansion Diseases

1 Oct 1998

Abstract excerpt

An increasing number of neurodegenerative diseases, including Huntington's disease (HD), have been found to be caused by a CAG/polyglutamine expansion. We have generated a mouse model of HD by the introduction of exon 1 of the human HD gene carrying highly expanded CAG repeats into the mouse germ line. These mice develop a progressive neurological phenotype. Neuronal intranuclear inclusions (NII) that are...

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PMID: 9804379
DOI: 10.1111/j.1750-3639.1998.tb00196.x

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