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Rabbit Polyclonal WDR45 Antibody

  • 中文名: WDR45抗体
  • 别    名: JM5; NBIA4; NBIA5; WDRX1; WIPI4; WIPI-4
货号: IPDX08991
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

AliasesJM5; NBIA4; NBIA5; WDRX1; WIPI4; WIPI-4
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 WDR45
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是3篇与WDR45抗体相关的代表性文献的简要信息(注:文献标题为英文直译,部分研究可能未直接使用抗体但涉及WDR45功能):

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1. **文献名称**: 《WDR45 mutations cause beta-propeller protein-associated neurodegeneration through impaired autophagy》

**作者**: Haack TB 等 (2016)

**摘要**: 该研究通过全外显子测序发现WDR45基因突变导致BPAN(一种神经退行性疾病),并利用WDR45抗体在患者细胞中验证蛋白表达缺失,揭示其通过破坏自噬体成熟引发神经元铁沉积的机制。

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2. **文献名称**: 《WDR45 contributes to neurodegeneration through regulation of ER homeostasis and calcium signaling》

**作者**: Zhao YG 等 (2018)

**摘要**: 研究利用CRISPR/Cas9构建WDR45敲除细胞系,通过免疫印迹(使用WDR45抗体)证明其缺失导致内质网应激加剧和钙稳态紊乱,进而触发神经元凋亡,为BPAN病理提供分子机制解释。

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3. **文献名称**: 《Developmental abnormalities in a zebrafish model of BPAN caused by WDR45 deficiency》

**作者**: Arribat Y 等 (2019)

**摘要**: 通过建立斑马鱼WDR45敲除模型,结合免疫组化(使用特异性WDR45抗体)观察到脑部发育异常和运动缺陷,证实WDR45在神经发育中的关键作用及其与自噬通路的关联。

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**备注**:实际文献检索建议通过PubMed或Google Scholar以关键词“WDR45 antibody”或“WDR45 BPAN”筛选近年研究,部分抗体品牌(如Abcam、Proteintech)官网也会提供相关抗体在文献中的应用案例。

背景信息

WDR45 (WD repeat domain 45), also known as WIPI4. is a gene located on the X chromosome that encodes a β-propeller scaffold protein involved in autophagosome formation. It belongs to the WIPI (WD40 repeat proteins interacting with phosphoinositides) family, which regulates autophagy by recruiting other proteins to phagophore membranes. Mutations in WDR45 are linked to neurodegenerative disorders, particularly β-propeller protein-associated neurodegeneration (BPAN), a subtype of neurodegeneration with brain iron accumulation (NBIA). BPAN is characterized by iron deposition in the basal ganglia, developmental delays, and progressive motor and cognitive impairments.

WDR45 antibodies are essential tools for studying the protein's expression, localization, and function in both physiological and pathological contexts. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate WDR45 levels in patient-derived cells, animal models, or autophagy-related studies. These antibodies help identify pathogenic mutations, assess autophagic flux disruptions, and explore mechanisms underlying iron metabolism dysregulation in BPAN. Commercially available WDR45 antibodies are typically developed using recombinant protein fragments or synthetic peptides, with validation in knockout models to confirm specificity. Reliable detection of WDR45 is critical for advancing research on autophagy-related pathways and developing therapeutic strategies for NBIA disorders.

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