Article
PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins.
The Journal of cell biology - 3 Jul 2006
Janer Alexandre, Martin Elodie, Muriel Marie-Paule, Latouche Morwena, Fujigasaki Hiroto, Ruberg Merle, Brice Alexis, Trottier Yvon, Sittler Annie
Abstract excerpt
The pathogenesis of spinocerebellar ataxia type 7 and other neurodegenerative polyglutamine (polyQ) disorders correlates with the aberrant accumulation of toxic polyQ-expanded proteins in the nucleus. Promyelocytic leukemia protein (PML) nuclear bodies are often present in polyQ aggregates, but their relation to pathogenesis is unclear. We show that expression of PML isoform IV leads to the formation of distinct...
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