Article
17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy.
Neurobiology of disease - 1 Jan 2011
Rusmini Paola, Simonini Francesca, Crippa Valeria, Bolzoni Elena, Onesto Elisa, Cagnin Monica, Sau Daniela, Ferri Nicola, Poletti Angelo
Abstract excerpt
Several types of motorneuron diseases are linked to neurotoxic mutant proteins. These acquire aberrant conformations (misfolding) that trigger deleterious downstream events responsible for neuronal dysfunction and degeneration. The pharmacological removal of misfolded proteins might thus be useful in these diseases. We utilized a peculiar motorneuronal disease model, spinobulbar muscular atrophy (SBMA), in which...
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