DATASHEET
Host:
Rabbit
Target Protein:
Gigaxonin
Immunogen Range:
351-450/597
Clonality:
Polyclonal
Isotype:
IgG
Source:
KLH conjugated synthetic peptide derived from human Gigaxonin
Purification:
Purified by Protein A.
Storage Buffer:
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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.
Conjugation:
APC
Excitation/ Emission:
650nm/660nm
Size:
100ul
Concentration:
1ug/ul
Applications:
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)
68
Rat
Predicted Cross Reactive Species:
Human
Mouse
Cow
Sheep
Pig
Horse
Rabbit
For research use only. Not intended for diagnostic or therapeutic use.