A protein (597 aa) that is thought to be important in the crosstalk between the intermediate filament and microtubule networks. One of the cytoskeletal BTB/kelch (broad-complex, tramtrack, and bric-a-brac) repeat family. It controls protein degradation and is essential for neuronal function and survival. Gigaxonin binds to the ubiquitin-activating enzyme E1 and interacts with microtubule-associated protein-1B. Mutations lead to giant axonal neuropathy.
Subjects: Medicine and Health.