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GENES & DEVELOPMENT 20:2183-2192, 2006
©2006 by Cold Spring Harbor Laboratory Press; ISSN 0890-9369/ $5.00
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REVIEW

Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle

Brigit E. Riley1,3,4 and Harry T Orr1,2,3,5

1 Department of Biochemistry, Molecular Biology and Biophysics, University of Minnesota, Minneapolis, Minnesota 55455, USA; 2 Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota 55455, USA; 3 Institute of Human Genetics, University of Minnesota, Minneapolis, Minnesota 55455, USA

The polyglutamine disorders are a class of nine neuro-degenerative disorders that are inherited gain-of-function diseases caused by expansion of a translated CAG repeat. Even though the disease-causing proteins are widely expressed, specific collections of neurons are more susceptible in each disease, resulting in characteristic patterns of pathology and clinical symptoms. One hypothesis poses that altered protein function is fundamental to pathogenesis, with protein context of the expanded polyglutamine having key roles in disease-specific processes. This review will focus on the role of the disease-causing polyglutamine proteins in gene transcription and the extent to which the mutant proteins induce disruption of transcription.

[Keywords: Gene expression; neurodegenerative disease; polyglutamine; transcription; trinucleotide repeats]

4 Present address: Department of Biology, Stanford University, Stanford, CA 94305.

5 Corresponding author. E-MAIL orrxx002{at}umn.edu; FAX (612) 626-7031.

Article is online at http://www.genesdev.org/cgi/doi/10.1101/gad.1436506.


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