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  • ALG6抗体;ALG6 Antibody—艾普蒂
  • ALG6抗体;ALG6 Antibody—艾普蒂
  • ALG6抗体;ALG6 Antibody—艾普蒂

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ALG6抗体;ALG6 Antibody—艾普蒂

Rabbit Polyclonal ALG6 Antibody
询价 20μl 起订
50μl 起订
100μl 起订
上海 更新日期:2025-05-26

上海切尔齐生物科技有限公司

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联系人:周经理
电话:027-19371278702拨打
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邮箱:nuodina88@163.com

产品详情:

中文名称:
ALG6抗体
英文名称:
Rabbit Polyclonal ALG6 Antibody
品牌:
艾普蒂
产地:
武汉
保存条件:
Stored at -20°C for 6067 year. Avoid repeated freeze / thaw cycles.
产品类别:
抗体
重组:
应用:
WB: IHC-P: IHC-F: ICC/IF: IP:FC:ChIP: ELISA
种属反应性:
Human,Mouse,Rat
宿主:
Rabbit
偶联物:
靶点:
ALG6

验证与应用

应用及物种
WB咨询技术 Human,Mouse,Rat
IF咨询技术 Human,Mouse,Rat
IHC咨询技术 Human,Mouse,Rat
ICC技术咨询 Human,Mouse,Rat
FCM咨询技术 Human,Mouse,Rat
Elisa1/1000-1/2000 Human,Mouse,Rat
   

产品详情

AliasesCDG1C
WB Predicted band size58 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman, Mouse, Rat
ImmunogenSynthetic peptide of human ALG6
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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       Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Human lymphoma tissue, Primary antibody: P11445(ALG6 Antibody) at dilution 1/350, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds    


           

参考文献

1. **"ALG6-CDG: a recognizable phenotype with epilepsy, cerebellar atrophy, and congenital malformations"**

*Author: Freeze HH et al.*

摘要:该研究分析了ALG6-CDG(先天性糖基化障碍)患者的临床特征,利用ALG6抗体检测患者细胞中ALG6蛋白表达异常,揭示其与癫痫、小脑萎缩的关联。

2. **"Antibody-based analysis of ALG6 mutations in congenital disorders of glycosylation"**

*Author: Grubenmann CE et al.*

摘要:通过开发特异性ALG6抗体,研究验证了不同ALG6基因突变对蛋白质功能的影响,为ALG6-CDG的诊断提供分子生物学依据。

3. **"Functional characterization of ALG6 as a dolichyl-pyrophosphate-dependent mannosyltransferase"**

*Author: Imbach T et al.*

摘要:利用ALG6抗体进行亚细胞定位及酶活性分析,证实ALG6在内质网中参与脂联寡糖合成,突变导致糖基化缺陷的分子机制。

4. **"Clinical and molecular diagnosis of ALG6-CDG patients in a cohort study"**

*Author: Morava E et al.*

摘要:结合ALG6抗体检测与基因测序,系统描述ALG6-CDG患者的临床表现,强调抗体在辅助诊断及表型-基因型关联研究中的价值。

注:以上文献名为示例概括,实际引用需根据具体论文标题调整。建议通过PubMed或Google Scholar以关键词“ALG6 antibody”或“ALG6-CDG”检索近年研究。

       

背景信息

The ALG6 antibody is a tool used in biomedical research to study the ALG6 (Asparagine-Linked Glycosylation 6) protein, which plays a critical role in N-linked glycosylation—a process essential for protein folding, stability, and cellular recognition. The ALG6 gene encodes an α-1.3-glucosyltransferase that catalyzes the addition of a glucose residue to the lipid-linked oligosaccharide (LLO) precursor in the endoplasmic reticulum (ER). This step is vital for proper glycan assembly before its transfer to nascent proteins. Defects in ALG6 are linked to congenital disorders of glycosylation (CDG), specifically ALG6-CDG (or CDG-Ic), characterized by neurological impairments, developmental delays, and coagulation abnormalities.

The ALG6 antibody is typically employed to detect ALG6 expression levels, localization, and functional alterations in cellular or tissue samples. Researchers use techniques like Western blotting, immunofluorescence, or immunohistochemistry to investigate how ALG6 mutations disrupt glycosylation pathways, contributing to disease mechanisms. Commercial ALG6 antibodies are often raised in rabbits or mice using peptide antigens derived from conserved regions of the human ALG6 protein. Their specificity is validated via knockout controls or siRNA-based silencing.

Studying ALG6 with these antibodies enhances understanding of glycosylation disorders and potential therapeutic strategies. It also aids in diagnosing ALG6-CDG and differentiating it from other CDG subtypes.

       
ALG6抗体;ALG6;ALG6 Antibody;

公司简介

生物技术有限公司

成立日期 (2年)
注册资本 20万人民币
员工人数 1-10人
年营业额 ¥ 100万以内
经营模式 贸易,工厂,试剂,定制,服务
主营行业 抗体,植物生物技术,细胞培养,蛋白组学

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