YRP11030-01 [Polyclonal Antibody]
Gigaxonin Rabbit Polyclonal Antibody
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Species:   Rabbit
Applications:   IHC IF
Immunogen Range:   KLH conjugated synthetic peptide derived from human Gigaxonin
Clonality:   Polyclonal Antibody
Isotype:   IgG
GENE ID:  
Swiss Prot:  
Synonyms:   FLJ38059, GAN, GAN1, Kelch-like protein 16, giant axonal neuropathy, KLHL16, GAN_HUMAN.
Purification:   Purified by Protein A.
Storage:   Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months
Background:   Gigaxonin, also refered to as giant axonal neuropathy, GAN1, or KLHL16, controls protein degradation and is essential for neuronal function and survival. Gigaxonin is a member of the cytoskeletal BTB/kelch repeat family and influences cytoskeletal organization and dynamics, playing a large role in neurofilament architecture. The amino terminal BTB domain of gigaxonin binds to the ubiquitin-activating enzyme E1, while the carboxy-terminal kelch repeat domain interacts directly with the light chain of microtubule-associated protein 1B (MAP1B), and tags it for degredation. Overexpression of MAP1B may lead to neuronal cell death, whereas a reduction of MAP1B significantly improves the survival rate of neurons. Mutations in the Gigaxonin gene result in human giant axonal neuropathy (GAN), an autosomal recessive neurodegenerative disorder characterized by axonal degeneration caused by cytoskeletal abnormalities, including accumulated intermediate filaments.
Caculated MW:   /
Observed MW:   Refer to Figures
Applications:   IHC 1:100-1:500
IF 1:50-1:200
Reacitivity:   Human, Mouse, Rat
For research use only. Not intended for diagnostic or therapeutic use!
Additional information