N-(4-Bromophenyl)-3-[[(4-bromophenyl)amino]sulfonyl] benzamide is the first inhibitor of aggregation of the polyglutamine (polyQ) sequence of the Huntington′s Disease protein (huntingtin).
N-(4-Bromophenyl)-3-[[(4-bromophenyl)amino]sulfonyl]benzamide is the first inhibitor of aggregation of the polyglutamine (polyQ) sequence of the Huntington′s Disease protein (huntingtin); polyQ aggregation is neurotoxic, and inhibitors of aggregation may be therapeutic in HD and other polyQ diseases; C2-8 inhibited aggregation in HD PC12 cells with an IC50 of 50nM; when administered in food, suppressed neurodegeneration in vivo in the Drosophila HD model.
Proceedings of the National Academy of Sciences of the United States of America, 102(3), 892-897 (2005-01-12)
Polyglutamine (polyQ) disorders, including Huntington's disease (HD), are caused by expansion of polyQ-encoding repeats within otherwise unrelated gene products. In polyQ diseases, the pathology and death of affected neurons are associated with the accumulation of mutant proteins in insoluble aggregates.
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