H. H. Kampinga

Tertiary structure and conformational dynamics of the anti-amyloidogenic chaperone DNAJB6b at atomistic resolution

This study reveals the tertiary structure and conformational dynamics of DNAJB6b at atomistic resolution, showing it cycles between three states and has an autoinhibitory mechanism linked to disease mutations.

DNAJB8 oligomerization is mediated by an aromatic-rich motif that is dispensable for substrate activity

This paper identifies an aromatic-rich motif in DNAJB8 that mediates oligomerization and shows that disrupting this motif affects oligomerization but not substrate activity.

Nuclear poly-glutamine aggregates rupture the nuclear envelope and hinder its repair

Nuclear poly-glutamine aggregates cause nuclear envelope ruptures that impair repair, contributing to Huntington's disease pathology.

In vivo suppression of polyglutamine aggregation via co-condensation of the molecular chaperone DNAJB6

This study shows DNAJB6 suppresses polyglutamine aggregation in vivo through co-condensation with mobile polyQ assemblies, preventing their conversion to solid aggregates.

Replicative aging impedes stress-induced assembly of a key human protein disaggregase

This study identifies a stress-induced trimeric protein disaggregase in human cells that collapses during replicative aging, contributing to pathophysiological consequences.

Salt Inducible Kinase activation and IRE1-dependent intracellular ATP depletion to form Sec bodies in Drosophila cells.

This study identifies two pathways, SIK activation by salt stress and IRE1-dependent ATP depletion, that together induce Sec body formation in Drosophila cells under amino-acid starvation.

DNAJB chaperones inhibit aggregation of destabilised proteins via a C-terminal region distinct from that used to prevent amyloid formation

This study shows DNAJB chaperones inhibit FlucDM aggregation via proteasome-mediated degradation, using a C-terminal region distinct from that preventing amyloid formation.