Article
Small heat-shock protein HSPB1 mutants stabilize microtubules in Charcot-Marie-Tooth neuropathy.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 26 Oct 2011
Almeida-Souza Leonardo, Asselbergh Bob, d'Ydewalle Constantin, Moonens Kristof, Goethals Sofie, de Winter Vicky, Azmi Abdelkrim, Irobi Joy, Timmermans Jean-Pierre, Gevaert Kris, Remaut Han, Van Den Bosch Ludo, Timmerman Vincent, Janssens Sophie
Abstract excerpt
Mutations in the small heat shock protein HSPB1 (HSP27) are causative for Charcot-Marie-Tooth (CMT) neuropathy. We previously showed that a subset of these mutations displays higher chaperone activity and enhanced affinity to client proteins. We hypothesized that this excessive binding property might cause the HSPB1 mutant proteins to disturb the function of proteins essential for the maintenance or survival of...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
