Polyglutamine diseases: Protein cleavage and aggregation

Huda Y. Zoghbi, Harry T. Orr

Research output: Contribution to journalReview articlepeer-review

95 Scopus citations


Neuronal aggregates of the disease-causing protein, often in the nucleus of affected cells, are a pathological hallmark of the neurodegenerative diseases known as polyglutamine disorders. It was suggested that these nuclear aggregates are the cause of these disorders. However, recent evidence suggests that the aggregates, in fact, are not the pathogenic basis and, instead, may play a role in sequestration of the pathogenic protein.

Original languageEnglish (US)
Pages (from-to)566-570
Number of pages5
JournalCurrent opinion in neurobiology
Issue number5
StatePublished - Oct 1 1999


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