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网站主页 PHYH antibody PHYH抗体
  • PHYH抗体—艾普蒂
  • PHYH抗体—艾普蒂
  • PHYH抗体—艾普蒂

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PHYH抗体—艾普蒂

Rabbit Polyclonal PHYH 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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联系人：周经理
电话：027-19371278702拨打
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邮箱：ruihening88@163.com

产品详情:

中文名称：
PHYH抗体
英文名称：
Rabbit Polyclonal PHYH Antibody
品牌：
艾普蒂
产地：
武汉
保存条件：
Stored at -20°C for 4264 year. Avoid repeated freeze / thaw cycles.
产品类别：
抗体
重组：
否
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：
无
靶点：
PHYH

验证与应用

应用及物种
WB1/500-1/2000 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
Elisa1/2000-1/5000 Human,Mouse,Rat
   

产品详情

AliasesRD; LN1; PAHX; LNAP1; PHYH1
WB Predicted band size39 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
ImmunogenFusion protein of human PHYH
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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       Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane 1-2: A549 cells, mouse liver tissue, Primary antibody: P08621(PHYH Antibody) at dilution 1/300, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 1 minute    


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       The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P08621(PHYH Antibody) at dilution 1/20. (Original magnification: ×200)    


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       The image is immunohistochemistry of paraffin-embedded Human breast cancer tissue using P08621(PHYH Antibody) at dilution 1/20. (Original magnification: ×200)    


           

参考文献

以下是关于PHYH抗体的3篇参考文献，涵盖其功能、疾病关联及实验应用：

1. **文献名称**：*Phytanoyl-CoA hydroxylase deficiency—the enzyme defect in Refsum's disease*

**作者**：Jansen, G.A., et al.

**摘要**：该研究首次明确PHYH(植烷酰-CoA羟化酶)缺陷是Refsum病的致病原因，通过患者细胞实验证实PHYH酶活性缺失，并利用特异性抗体检测其蛋白表达异常。

2. **文献名称**：*Identification of PAHX as a Refsum disease gene by combined analysis of homozygosity mapping and metabolic function*

**作者**：Ferdinandusse, S., et al.

**摘要**：通过基因定位和功能分析，验证PHYH(PAHX)基因突变导致Refsum病，研究中使用抗体验证突变对蛋白稳定性的影响，揭示酶活性丧失机制。

3. **文献名称**：*Phytanoyl-CoA α-hydroxylase is not essential for degradation of phytanic acid in mice*

**作者**：Mihalik, S.J., et al.

**摘要**：通过PHYH基因敲除小鼠模型，探究植烷酸代谢途径，利用抗体检测PHYH蛋白缺失，发现其他代偿途径可能参与植烷酸降解。

4. **文献名称**：*Metabolic aspects of peroxisomal disorders in fatty acid β-oxidation*

**作者**：Wanders, R.J.A., et al.

**摘要**：综述中讨论PHYH在过氧化物酶体脂肪酸氧化中的作用，强调抗体的应用在疾病诊断中的作用，如Western blot和免疫荧光分析患者样本。

以上文献均涉及PHYH抗体在疾病机制、酶功能验证及诊断中的关键应用。

       

背景信息

The PHYH antibody targets the phytanoyl-CoA hydroxylase (PHYH) enzyme, encoded by the PHYH gene, which plays a critical role in lipid metabolism within peroxisomes. PHYH catalyzes the alpha-hydroxylation of phytanoyl-CoA, a key step in the breakdown of phytanic acid, a branched-chain fatty acid derived from dietary sources. Mutations in PHYH are linked to Refsum disease, a rare autosomal recessive disorder characterized by phytanic acid accumulation, leading to neurological, visual, and cardiac impairments.

PHYH antibodies are essential tools in studying peroxisomal disorders, particularly Refsum disease. They enable detection and quantification of PHYH protein expression in tissues or cell cultures, aiding in diagnostic assays and mechanistic research. These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess PHYH localization, expression levels, and potential dysfunction in disease models.

Additionally, PHYH antibodies contribute to understanding broader peroxisomal functions, including fatty acid alpha-oxidation pathways and their interplay with cellular metabolism. Their specificity and validation (e.g., knockout controls) ensure reliability in both basic research and clinical applications. By elucidating PHYH's role in health and disease, these antibodies support therapeutic development and biomarker discovery for peroxisome-related disorders.

       
PHYH抗体;PHYH;PHYH 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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