Cytoplasmic Penetration and Persistent Infection of Mammalian Cells by Polyglutamine Aggregates

摘要: Sequence-specific nucleated protein aggregation is closely linked to the pathogenesis of most neurodegenerative diseases and constitutes molecular basis prion formation. Here we report that fibrillar polyglutamine peptide aggregates can be internalized by mammalian cells in culture where they gain access cytosolic compartment become co-sequestered aggresomes together with components ubiquitin-proteasome system cytoplasmic chaperones. Remarkably, these are able selectively recruit soluble proteins which share homologous but not heterologous amyloidogenic sequences, confer a heritable phenotype on expressing from chromosomal locus.