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

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.

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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.

Perspectives