Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle

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