DNAJB6, a Key Factor in Neuronal Sensitivity to Amyloidogenesis

Arun Thiruvalluvan, Eduardo P. de Mattos, Jeanette F. Brunsting, Rob Bakels, Despina Serlidaki, Lara Barazzuol, Paola Conforti, Azra Fatima, Seda Koyuncu, Elena Cattaneo, David Vilchez, Steven Bergink, Erik H. W. G. Boddeke, Sjef Copray, Harm H. Kampinga*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

Thiruvalluvan et al. show that, upon differentiation, the cellular chaperone network is rewired. This includes a downregulation of the Hsp70 co-chaperone DNAJB6. High levels of DNAJB6 keep patient-derived neuronal progenitors resistant to the formation of amyloids. The sole knockdown of DNAJB6 in progenitors induces spontaneous aggregation of endogenously expressed polyglutamine proteins. Inversely, low levels of DNAJB6 in neurons render them hypersensitive to amyloid formation, which can be restored by re-elevating DNAJB6 expression levels.

Original languageEnglish
Pages (from-to)346-+
Number of pages22
JournalMolecular Cell
Volume78
Issue number2
Early online date2020
DOIs
Publication statusPublished - 16-Apr-2020

Keywords

  • HEAT-SHOCK PROTEINS
  • POLYGLUTAMINE AGGREGATION
  • HUNTINGTONS-DISEASE
  • ASYMMETRIC INHERITANCE
  • MOLECULAR CHAPERONES
  • ANDROGEN RECEPTOR
  • DAMAGED PROTEINS
  • RICH REGION
  • CELL LINES
  • MUTATIONS

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