巨轴索神经病蛋白GAN抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 巨轴索神经病蛋白GAN抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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, 巨轴索神经病蛋白GAN抗体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 巨轴索神经病蛋白GAN抗体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.

巨轴索神经病蛋白GAN抗体Also known as:

FLJ38059; GAN; GAN1; Kelch-like protein 16; giant axonal neuropathy; KLHL16; GAN_HUMAN.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human Gigaxoni.

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Predicted Molecular Weight: 68kDa.

巨轴索神经病蛋白GAN抗体Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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