Kelch-like protein, gigaxonin <p>Kelch is a 50-residue motif, named after the Drosophila mutant in which it was first identified [<cite idref="PUB00000885"/>]. This sequence motif represents one beta-sheet blade, and several of these repeats can associate to form a beta-propeller.</p><p>This group represents a kelch-like protein, gigaxonin type. Giant axonal neuropathy (GAN) is a devastating sensory and motor neuropathy caused by mutations in the GAN gene, which encodes the ubiquitously expressed protein gigaxonin. Gigaxonin binds to the ubiquitin-activating enzyme E1 through its N-terminal BTB domain and its six C-terminal kelch repeat domain interacts directly with the light chain (LC) of microtubule-associated protein 1B (MAP1B) [<cite idref="PUB00043351"/>, <cite idref="PUB00043352"/>].</p>