Kelch Domain of Gigaxonin Interacts with Intermediate Filament Proteins Affected in Giant Axonal Neuropathy

  • Bethany L. Johnson-Kerner, Alejandro Garcia Diaz, Sean Ekins, Hynek Wichterle
  • PLoS ONE, October 2015, Public Library of Science (PLoS)
  • DOI: 10.1371/journal.pone.0140157

What is it about?

Giant Axonal Neuropathy (GAN) is a rare disease that leads to low levels of gigaxonin, leading to aggregates of intermediate filaments. This study describes a proteomic approach to understand what Gigaxonin binds to. The kelch domian was found to be important along with HSP90 and Myc binding to the BTB domian.

Why is it important?

This work could lead to targets for drug discovery to treat this rare disease - by clearing the accumulated intermediate filaments that are present in GAN patients.


Dr Sean Ekins
Collaborations in Chemistry

My contribution to this highly collaborative study was the Ingenuity Pathways Analysis of the proteomic data.

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The following have contributed to this page: Dr Sean Ekins