Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle

B. E. Riley
2006 Genes & Development  
The polyglutamine disorders are a class of nine neurodegenerative 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
more » ... f the expanded polyglutamine having key roles in diseasespecific 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. 4 Present address: ; FAX (612) 626-7031. Article is online at http://www.genesdev.org/cgi/
doi:10.1101/gad.1436506 pmid:16912271 fatcat:rd563sxrsbhffeykibddelcpbi