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...
Topics
- Animals
- Animals, Genetically Modified
- Bulbo-Spinal Atrophy, X-Linked
- Cell Line
- Disease Models, Animal
- Drosophila melanogaster
- Humans
- Intracellular Space
- Intranuclear Inclusion Bodies
- Ligands
