跳转至内容
Merck
CN

SAB4200104

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)

登录 查看组织和合同定价。

选择尺寸

关于此项目

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
IF, IP, WB
Citations:
7

主要文件

查看所有文档
技术服务
需要帮助？我们经验丰富的科学家团队随时乐意为您服务。
让我们为您提供帮助
技术服务
需要帮助？我们经验丰富的科学家团队随时乐意为您服务。
让我们为您提供帮助

biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen ~70 kDa

species reactivity

mouse, human, rat

concentration

~1.5 mg/mL

technique(s)

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 accession no.

Q9H2C0

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

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

General description

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

Application

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

Biochem/physiol Actions

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.

Physical form

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

Disclaimer

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.

未找到合适的产品？  

试试我们的产品选型工具.

存储类别

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable

法规信息

新产品
此项目有

历史批次信息供参考:

分析证书(COA)

Lot/Batch Number
030M4759

没有发现合适的版本？

如果您需要特殊版本，可通过批号或批次号查找具体证书。

搜索COA

已有该产品？

在文件库中查找您最近购买产品的文档。

访问文档库

Electronic medical records and genomics (eMERGE) network exploration in cataract: Several new potential susceptibility loci
Ritchie MD
Molecular Vision, 20, 1281-1295 (2014)
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)
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
查看所有相关文献

相关内容

我们的科学家团队拥有各种研究领域经验，包括生命科学、材料科学、化学合成、色谱、分析及许多其他领域.

联系客户支持