Polyglutamine oligomers

Preventing polyglutamine repeat oligomerization reverses symptoms of Huntington's chorea.

Jonathan Weitzman(jonathanweitzman@hotmail.com)
Jan 22, 2003

Expansion of trinucleotide repeats, such as CAG that encodes for polyglutamine, is associated with a number of neurodegenerative disorders. Expanded polyglutamine fragments form aggregates that have been proposed to be linked to neurodegeneration. In the January 23 Nature, Ivelisse Sanchez and colleagues provide strong evidence that polyglutamine oligomerization leads to chronic cytotoxicity (Nature, 421:373-379, January 23, 2003).

Sanchez et al. screened a range of anti-amyloid compounds and observed that the azo-dye Congo red blocked polyglutamine-induced cell death. Congo red also inhibited ATP depletion and caspase activation. The Congo red dye can block polyglutamine oligomerization and disrupt preformed aggregates.

They then tested the effects of Congo red in the R62 model of Huntington's chorea and found that it induced the clearance of aggregates and improved survival, weight loss and motor function.

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