巨轴索神经病蛋白GAN抗体英文名称 Anti-Gigaxonin
中文名称 巨轴索神经病蛋白GAN抗体
别 名 FLJ38059; GAN; GAN1; Kelch-like protein 16; giant axonal neuropathy; KLHL16; GAN_HUMAN.
浓 度 1mg/1ml
规 格 0.2ml/200μg
纯化的抗体可通过不同的途径获取,有些巨轴索神经病蛋白GAN抗体可通过下述方法制备或从商家购买。从商家购买的抗体,通常附有正确的储存方法。
1)工作液应在4℃下融化并存放,可能稳定达数月。
2)如果没有特殊原因而避免使用叠氮钠,亦可加入叠氮钠,浓度为0.02%。将纯化的抗体样本分装成合适的体积,于-20℃保存。
3)纯化的抗体溶液应以较高的浓度(如lmg/m1)在中性pH下保存。:常用的抗体储存浓度高达l0mg/ml。较低浓度的抗体冻存前应浓缩。所有标准的浓缩方法(如超滤法),皆可使用。还有一个简单的方法是用蛋白A或蛋白G亲和柱来浓缩溶液。如果纯化的抗体不是用于标记,可将它们以较低浓度储存于加有1%BSA的溶液中。
4)经纯化制备的抗体在常用的缓冲液中是稳定的。其DH应保持在中性左右。如果pH在7-8之间,即使保存多年,对抗体也无损害。多数情况下,盐浓度适于保持在0-150mmol/L之间,但在长期存放的抗体中,盐溶液浓度高达500mmol/L时,对巨轴索神经病蛋白GAN抗体能有损害。如果没有其他说明.律议用PBS或50mmol/LTris(DH8.0)溶液长期存放抗体。
抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep
产品类型 一抗
研究领域 神经生物学 信号转导 细胞粘附分子 细胞骨架
蛋白分子量 predicted molecular weight: 68kDa
性 状 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human Gigaxonin
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
产品应用 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
(石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
巨轴索神经病蛋白GAN抗体产品介绍 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.
Function : 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
Subunit : 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).
Subcellular Location : Cytoplasmic; Cytoskeleton.
Tissue Specificity : Expressed in brain, heart and muscle.
Post-translational modifications : Ubiquitinated by E3 ubiquitin ligase complex formed by CUL3 and RBX1 and probably targeted for proteasome-independent degradation.
DISEASE : Defects in GAN are the cause of giant axonal neuropathy (GAN) [MIM:256850]. GAN is a severe autosomal recessive sensorimotor neuropathy affecting both the peripheral nerves and the central nervous system. It is characterized by neurofilament accumulation, leading to segmental distention of axons.
Similarity : Contains 1 BACK (BTB/Kelch associated) domain.
Contains 1 BTB (POZ) domain.
Contains 6 Kelch repeats.
Database links : UniProtKB/Swiss-Prot: Q9H2C0.1