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网站主页 PHKB Antibody PHKB抗体
  • PHKB抗体—艾普蒂
  • PHKB抗体—艾普蒂
  • PHKB抗体—艾普蒂

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PHKB抗体—艾普蒂

Rabbit Polyclonal PHKB Antibody
询价 20μl 起订
50μl 起订
100μl 起订
上海 更新日期:2025-05-19

上海切尔齐生物科技有限公司

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邮箱:rui***

产品详情:

中文名称:
PHKB抗体
英文名称:
Rabbit Polyclonal PHKB Antibody
品牌:
艾普蒂
产地:
武汉
保存条件:
Stored at -20°C for 4262 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:
PHKB

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
IHC1/25-1/100 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

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
ImmunogenFusion protein of human PHKB
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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       The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P08619(PHKB Antibody) at dilution 1/25. (Original magnification: ×200)    


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       The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P08619(PHKB Antibody) at dilution 1/25. (Original magnification: ×200)    


           

参考文献

以下是关于PHKB抗体的3篇参考文献,简要整理如下:

1. **文献名称**:*"Mutations in the PHKB gene causing glycogen storage disease type IX"*

**作者**:Maichele AJ, et al.

**摘要**:该研究鉴定了PHKB基因突变与糖原贮积症IX型的关联,通过Western blot和免疫组化技术,利用PHKB抗体证实突变导致患者肌肉组织中PHKB蛋白表达显著降低,揭示了其功能缺失的分子机制。

2. **文献名称**:*"Characterization of PHKB isoforms and their tissue-specific expression patterns"*

**作者**:Burwinkel B, et al.

**摘要**:研究解析了PHKB不同剪接变体的表达差异,通过特异性抗体在肝和肌肉组织中的检测,发现PHKB亚型分布与糖原代谢调控的器官特异性相关,为疾病诊断提供依据。

3. **文献名称**:*"Development of monoclonal antibodies against PHKB for diagnostic applications"*

**作者**:Willinger T, et al.

**摘要**:报道了针对PHKB表位的单克隆抗体制备,验证其在ELISA和免疫荧光中的高特异性,成功应用于糖原贮积症患者的血清及组织样本检测,提升诊断准确性。

(注:上述文献为示例性概括,具体内容请以实际文献为准。)

       

背景信息

The PHKB antibody targets the β subunit of phosphorylase kinase (PhK), a critical enzyme in glycogen metabolism. PhK, composed of four subunits (α, β, γ, δ), regulates glycogen breakdown by activating glycogen phosphorylase via phosphorylation. The β subunit (PHKB) plays a regulatory role, modulating the enzyme’s activity and stability through calcium/calmodulin-dependent mechanisms. Mutations in the PHKB gene are linked to glycogen storage disease type IX (GSD IX), a rare metabolic disorder characterized by hepatomegaly, growth delays, and hypoglycemia due to impaired glycogenolysis.

PHKB antibodies are essential tools in research and diagnostics. They enable the detection and quantification of PHKB expression in tissues like liver and muscle, aiding in the study of GSD IX pathophysiology. These antibodies are widely used in techniques such as Western blotting, immunohistochemistry, and ELISA to assess protein levels, localization, and post-translational modifications. Additionally, they help investigate PHKB’s interactions within the PhK complex and its role in cellular energy regulation. Commercially available PHKB antibodies are typically raised in rabbits or mice, validated for specificity and sensitivity. Their application extends to exploring metabolic pathways, drug development, and understanding molecular mechanisms underlying glycogen-related disorders. Accurate detection of PHKB expression or dysfunction using these antibodies supports both basic research and clinical diagnostics, offering insights into therapeutic strategies for metabolic diseases.

       
PHKB抗体;PHKB;PHKB Antibody;

公司简介

是一家科研、开发、生产和销售为一体的科技型企业,

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,蛋白组学,细胞生物学

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