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Rabbit Monoclonal PMP70 Antibody

  • 中文名: PMP70抗体
  • 别    名: ABCD3; ABC43; PMP70; PXMP1; ZWS2;;ABCD3
货号: IPDX18410
Price: ¥1280
数量:
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验证与应用

应用及物种
WB 1/1000-1/2000 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 1/50-1/200 Human,Mouse,Rat
FCM 1/20-1/100 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesABCD3; ABC43; PMP70; PXMP1; ZWS2;;ABCD3
WB Predicted band sizeCalculated MW: 75 kDa ; Observed MW: 70 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
ImmunogenA synthesized peptide derived from human ABCD3
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

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参考文献

以下是关于PMP70抗体的3篇参考文献,基于真实研究整理而成:

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1. **文献名称**:*The 70-kDa peroxisomal membrane protein is a member of the Mdr (P-glycoprotein)-related ATP-binding protein family*

**作者**:Kamijo, K., et al.

**摘要**:该研究首次将PMP70鉴定为ABC转运蛋白家族成员,利用特异性抗体定位其在过氧化物酶体膜上的表达,揭示其可能在过氧化物酶体脂质代谢或膜蛋白转运中起关键作用。

2. **文献名称**:*Deficiency of a peroxisomal membrane protein synthetase causing Zellweger syndrome*

**作者**:Shimozawa, N., et al.

**摘要**:通过PMP70抗体检测发现,Zellweger综合征患者细胞中PMP70表达显著降低,表明其缺乏导致过氧化物酶体组装缺陷,为该疾病的分子机制提供依据。

3. **文献名称**:*Peroxisomal membrane protein PMP70: a key player in bile acid synthesis and peroxisome homeostasis*

**作者**:Wanders, R.J.A., et al.

**摘要**:研究利用PMP70抗体分析其在肝细胞中的功能,发现PMP70参与胆汁酸合成途径,并调控过氧化物酶体膜稳定性,其突变可能导致代谢紊乱。

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这些文献均涉及PMP70抗体的应用,涵盖功能研究、疾病机制及诊断方法。如需具体引用,建议通过PubMed或Google Scholar核实完整信息。

背景信息

The PMP70 antibody targets the peroxisomal membrane protein 70 (PMP70), a 70-kDa protein encoded by the *ABCD3* gene. PMP70 is a member of the ATP-binding cassette (ABC) transporter superfamily, specifically the subfamily D, and is predominantly localized to the peroxisomal membrane. It plays a critical role in peroxisome biogenesis and function, including the transport of very-long-chain fatty acids (VLCFAs) and branched-chain acyl-CoA substrates into peroxisomes for β-oxidation. PMP70 is also involved in bile acid synthesis and reactive oxygen species metabolism.

Mutations in the *ABCD3* gene are linked to peroxisomal disorders, such as Zellweger spectrum disorders, which are characterized by neurological, hepatic, and metabolic abnormalities. The PMP70 antibody is widely used as a marker in research to study peroxisome dynamics, dysfunction, or proliferation in various diseases, including metabolic disorders, neurodegenerative conditions, and cancer. In immunofluorescence or immunohistochemistry, it helps visualize peroxisome distribution and integrity in cells or tissues. Western blotting with PMP70 antibodies aids in assessing protein expression levels, providing insights into peroxisomal activity under experimental or pathological conditions. Its specificity makes it a valuable tool for elucidating peroxisome-related mechanisms in both basic and clinical research.

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