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Article

Progressive retinal degeneration and dysfunction in R6 Huntington's disease mice

6 Dec 2002

Abstract excerpt

Huntington's disease (HD) and spinocerebellar ataxia type 7 (SCA7) belong to a group of progressive neurodegenerative diseases caused by polyglutamine (polyQ) expansions. SCA7 is the only one to display degeneration in the retina, a tissue usually spared in HD. We previously described a SCA7 transgenic retinal model expressing mutant full length ataxin-7 in rod photoreceptors. These mice develop a severe and...

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PMID: 12471061
DOI: 10.1093/hmg/11.26.3351

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