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Merck
CN

SAB4200104

Sigma-Aldrich

Anti-Gigaxonin (N-terminal) antibody produced in rabbit

~1.5 mg/mL, affinity isolated antibody

别名:

Anti-GAN, Anti-GAN1, Anti-Giant axonal neuropathy 1, Anti-KLHL16 (kelch-like protein 16)

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关于此项目

UNSPSC代码:
12352203
NACRES:
NA.41

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生物来源

rabbit

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

表单

buffered aqueous solution

分子量

antigen ~70 kDa

种属反应性

mouse, human, rat

浓度

~1.5 mg/mL

技术

immunoprecipitation (IP): 15-30 μg using rat brain extracts (S1 fraction)
indirect immunofluorescence: 10-20 μg/mL using HeLa cells expressing human gigaxonin
western blot: 1-2 μg/mL using mouse brain extracts (S1 fraction), and 1.5-3 μg/mL using SH-SY5Y cell lysates
western blot: 1.5-3 μg/mL using SH-SY5Y cell lysates

UniProt登记号

Q9H2C0

运输

dry ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... GAN(8139)
mouse ... Gan(209239)
rat ... Gan(307893)

一般描述

Gigaxonin (GAN) is encoded by the gene mapped to human chromosome 16q23.2. The encoded protein is characterized with an N-terminal BTB (for broad-complex, ramtrack and bric a brac) domain followed by a six kelch repeats, which are predicted to adopt a β-propeller shape.8

应用

Anti-Gigaxonin (N-terminal) antibody produced in rabbit has been used in immunofluorescence and immunoblotting assay.

生化/生理作用

Gigaxonin (GAN) acts as a key regulator of intermediate filaments (IFs) and the microtubule-associated protein 1B (MAP1B) light chain degradation, which is required for neuronal survival. GAN contributes to neurofilament architecture. Additionally, it also influences the ubiquitination and degradation of some proteins. Mutation in the gene is associated with the development of giant axonal neuropathy.

外形

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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储存分类代码

10 - Combustible liquids

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

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分析证书(COA)

Lot/Batch Number
030M4759

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The gene encoding gigaxonin, a new member of the cytoskeletal BTB/kelch repeat family, is mutated in giant axonal neuropathy
Bomont P
Nature Genetics, 26, 370-374 (2000)
Electronic medical records and genomics (eMERGE) network exploration in cataract: Several new potential susceptibility loci
Ritchie MD
Molecular Vision, 20, 1281-1295 (2014)
Novel homozygous missense mutation in GAN associated with Charcot-Marie-Tooth disease type 2 in a large consanguineous family from Israel
Aharoni S
BMC Medical Genetics, 17, 82-82 (2016)
Rachel M Bailey et al.
Molecular therapy. Methods & clinical development, 9, 160-171 (2018-05-17)
An NIH-sponsored phase I clinical trial is underway to test a potential treatment for giant axonal neuropathy (GAN) using viral-mediated GAN gene replacement (https://clinicaltrials.gov/ct2/show/NCT02362438). This trial marks the first instance of intrathecal (IT) adeno-associated viral (AAV) gene transfer in humans.
Po-Han Chen et al.
JCI insight, 5(1) (2020-01-17)
Gigaxonin (also known as KLHL16) is an E3 ligase adaptor protein that promotes the ubiquitination and degradation of intermediate filament (IF) proteins. Mutations in human gigaxonin cause the fatal neurodegenerative disease giant axonal neuropathy (GAN), in which IF proteins accumulate
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