Article
B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy.
Journal of neuroscience research - 1 Aug 2010
Palazzolo Isabella, Nedelsky Natalia B, Askew Caitlin E, Harmison George G, Kasantsev Aleksey G, Taylor J Paul, Fischbeck Kenneth H, Pennuto Maria
Abstract excerpt
Expanded polyglutamine tracts cause neurodegeneration through a toxic gain-of-function mechanism. Generation of inclusions is a common feature of polyglutamine diseases and other protein misfolding disorders. Inclusion formation is likely to be a defensive response of the cell to the presence of unfolded protein. Recently, the compound B2 has been shown to increase inclusion formation and decrease toxicity of...
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