Anti-Gigaxonin Polyclonal Antibody is a Rabbit antibody targeting Gigaxonin. Anti-Gigaxonin Polyclonal Antibody can be used in IHC-P,IHC-Fr,IF.

IgG

Rat (predicted:Human,Mouse,Pig,Cow,Horse,Rabbit,Sheep)

IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500

Polyclonal

KLH conjugated synthetic peptide: human Gigaxonin

Human

GAN

Gigaxonin

Mutations in gigaxonin result in a sensory and motor neuropathy called Giant Axonal Neuropathy (GAN). Giant axonal neuropathy, a severe autosomal recessive sensorineural neuropathy affecting both the peripheral nerves and the central nervous system, is characterized by neurofilament accumulation, leading to segmental distention of axons. Gigaxonin is a member of the cytoskeletal BTB/kelch (Broad-Complex, Tramtrack and Bric a brac) repeat family. Gigaxonin contains an N-terminal BTB domain followed by 6 kelch repeats, which were predicted to adopt a beta-propeller shape. Gigaxonin controls protein degradation and is essential for neuronal function and survival. Substrate-specific adapter of an E3 ubiquitin-protein ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Controls degradation of TBCB. Controls degradation of MAP1B and MAP1S, and is critical for neuronal maintenance and survival