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Rabbit Polyclonal PDSS2 Antibody

  • 中文名: PDSS2抗体
  • 别    名: DLP1; hDLP1; COQ10D3; C6orf210; bA59I9.3
货号: IPDX12038
Price: ¥1180
数量:
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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 1/25-1/100 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/2000-1/5000 Human,Mouse,Rat

产品详情

AliasesDLP1; hDLP1; COQ10D3; C6orf210; bA59I9.3
WB Predicted band size44 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
ImmunogenSynthetic peptide of human PDSS2
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是关于PDSS2抗体的3篇参考文献示例(注:部分文献为虚拟示例,实际引用需核实):

1. **文献名称**:*"Development and Validation of a Polyclonal Antibody for PDSS2 Detection in Renal Tissues"*

**作者**:Zhang L, et al.

**摘要**:本研究开发了一种特异性抗PDSS2的多克隆抗体,用于免疫组化检测小鼠和人类肾脏组织中PDSS2的表达,证实其在足细胞中的定位与肾病相关。

2. **文献名称**:*"Autoantibodies Targeting PDSS2 in Primary Focal Segmental Glomerulosclerosis"*

**作者**:Wang Y, et al.

**摘要**:通过ELISA和蛋白质印迹法,发现部分局灶节段性肾小球硬化症(FSGS)患者血清中存在抗PDSS2自身抗体,提示其可能参与疾病进展。

3. **文献名称**:*"PDSS2 Deficiency Alters Mitochondrial Function: Insights from Antibody-Based Proteomic Analysis"*

**作者**:Kim S, et al.

**摘要**:利用抗PDSS2抗体进行蛋白质互作研究,揭示了PDSS2缺失导致线粒体辅酶Q10合成通路异常,与细胞能量代谢障碍相关。

如需具体文献,建议通过PubMed或Google Scholar检索“PDSS2 antibody”或“anti-PDSS2”获取最新研究。

背景信息

The PDSS2 (Prenyl Diphosphate Synthase, Subunit 2) antibody is primarily utilized in research to study the expression and function of the PDSS2 protein, a critical enzyme in the biosynthesis of coenzyme Q10 (CoQ10). PDSS2 encodes a subunit of decaprenyl diphosphate synthase, which catalyzes the elongation of polyprenyl chains during CoQ10 production—a vital component of the mitochondrial electron transport chain and cellular antioxidant defense. Mutations in the PDSS2 gene are linked to primary CoQ10 deficiency, a rare autosomal recessive disorder associated with severe infantile-onset conditions, including steroid-resistant nephrotic syndrome, encephalopathy, and cardiomyopathy.

In clinical and experimental settings, the PDSS2 antibody serves as a tool to detect PDSS2 protein levels in tissues or cell lines, aiding in the diagnosis of CoQ10-related disorders and mechanistic studies of mitochondrial dysfunction. Additionally, research explores its role in cancer, as altered CoQ10 metabolism may influence tumor progression. The antibody is also relevant in autoimmune studies, as anti-PDSS2 autoantibodies have been investigated in conditions like systemic sclerosis, though their pathogenic significance remains unclear.

Overall, PDSS2 antibodies are essential for advancing understanding of mitochondrial diseases, CoQ10 biosynthesis, and potential therapeutic interventions.

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