[HTML][HTML] Proteasome-mediated proteolysis of the polyglutamine-expanded androgen receptor is a late event in spinal and bulbar muscular atrophy (SBMA) …
EM Heine, TR Berger, A Pluciennik, CR Orr… - Journal of Biological …, 2015 - ASBMB
Proteolysis of polyglutamine-expanded proteins is thought to be a required step in the
pathogenesis of several neurodegenerative diseases. The accepted view for many
polyglutamine proteins is that proteolysis of the mutant protein produces a" toxic fragment"
that induces neuronal dysfunction and death in a soluble form; toxicity of the fragment is
buffered by its incorporation into amyloid-like inclusions. In contrast to this view, we show
that, in the polyglutamine disease spinal and bulbar muscular atrophy, proteolysis of the …
pathogenesis of several neurodegenerative diseases. The accepted view for many
polyglutamine proteins is that proteolysis of the mutant protein produces a" toxic fragment"
that induces neuronal dysfunction and death in a soluble form; toxicity of the fragment is
buffered by its incorporation into amyloid-like inclusions. In contrast to this view, we show
that, in the polyglutamine disease spinal and bulbar muscular atrophy, proteolysis of the …