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doi: 10.1016/s0896-6273(01)00435-4.
Qs in the nucleus
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- PMID: 11580886
- DOI: 10.1016/s0896-6273(01)00435-4
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Comment
Qs in the nucleus
Neuron.
.
Free article
Abstract
The polyglutamine diseases include at least nine neurodegenerative disorders. Accumulation of mutant protein with a toxic gain-in function in the nucleus appears to be the pathological basis of these diseases. In this issue of Neuron, La Spada et al. (2001) provide insight into the cell specificity of pathology for a polyglutamine disease by relating SCA7-induced retinal degeneration to a disruption of the photoreceptor-specific transcription factor CRX.
Comment on
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Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7.Neuron. 2001 Sep 27;31(6):913-27. doi: 10.1016/s0896-6273(01)00422-6. Neuron. 2001. PMID: 11580893
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