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| 规格： | 50ul | 产品价格： | ￥1180.0 |
|---|---|---|---|
| 规格： | 100ul | 产品价格： | ￥1980.0 |
| 规格： | 200ul | 产品价格： | ￥2800.0 |
| 产品编号 | bs-11025R |
| 英文名称 | Gigaxonin Rabbit pAb |
| 中文名称 | 巨轴索神经病蛋白GAN抗体 |
| 英文别名 | FLJ38059; GAN; GAN1; Kelch-like protein 16; giant axonal neuropathy; KLHL16; GAN_HUMAN. |
| 产品应用 | IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500 Not yet tested in other applications. |
| 交叉反应 | Rat (Human, Mouse, Pig, Cow, Horse, Rabbit, Sheep) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human Gigaxonin |
| 亚型 | IgG |
| 性状 | Liquid |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 理论分子量 | 68 kDa |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Neuroscience > Cell Adhesion Proteins > Cytoskeletal Proteins > Microtubules Neuroscience > Cell Type Marker > Neuron marker > Axon marker Signal Transduction > Cytoskeleton / ECM > Cytoskeleton > Intermediate Filaments > Class IV > Neurofilaments |
| 亚基 | Interacts with TBCB. Interacts with CUL3. Part of a complex that contains CUL3, RBX1 and GAN. Interacts (via BTB domain) with UBA1. Interacts (via Kelch domains) with MAP1B (via C-terminus) and MAP1S (via C-terminus). |
| 亚细胞定位 | Cytoplasmic; Cytoskeleton. |
| 组织特异性 | Expressed in brain, heart and muscle. |
| 翻译后修饰 | Ubiquitinated by E3 ubiquitin ligase complex formed by CUL3 and RBX1 and probably targeted for proteasome-independent degradation. |
| 相似性 | Contains 1 BACK (BTB/Kelch associated) domain. Contains 1 BTB (POZ) domain. Contains 6 Kelch repeats. |
| 功能 | 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 |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
| 应用 | 推荐稀释比例 |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {IF} | {1:100-500} |
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