Abstract:
:Polyglutamine diseases are a family of nine neurodegenerative disorders caused by expansion in different genes of a CAG triplet repeat stretch, which encodes an elongated polyglutamine tract. This polyglutamine tract is thought to confer a toxic gain of function to the bearing proteins, which leads to late onset and progressive loss of neurons in specific regions of the central nervous system. The mechanisms underlying specificity for neuronal vulnerability remain enigmatic. One explanation is that the polyglutamine tract is not the only determinant of neurodegeneration and that protein context and post-translational events may also be crucial for pathogenesis. Here, we review how post-translational modifications of the polyglutamine proteins contribute to modulate neurotoxicity.
journal_name
Hum Mol Genetjournal_title
Human molecular geneticsauthors
Pennuto M,Palazzolo I,Poletti Adoi
10.1093/hmg/ddn412subject
Has Abstractpub_date
2009-04-15 00:00:00pages
R40-7issue
R1eissn
0964-6906issn
1460-2083pii
ddn412journal_volume
18pub_type
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