| WB | 1/1000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Peroxisome biogenesis factor 1, Peroxin-1, Peroxisome biogenesis disorder protein 1, PEX1 |
| Entrez GeneID | 5189 |
| WB Predicted band size | 142.9kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human |
| Immunogen | This PEX1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 599-628 amino acids from the Central region of human PEX1. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3-4条关于PEX1抗体的参考文献及其简要摘要内容：
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1. **文献名称**：*Mutations in the PEX1 Gene and Their Use in Laboratory Diagnosis of Zellweger Syndrome*
**作者**：Braverman, N. et al.
**摘要**：研究通过Western blot和免疫荧光技术，利用PEX1特异性抗体分析患者细胞中PEX1蛋白的表达水平，发现PEX1基因突变导致齐薇格综合征(Zellweger syndrome)患者的蛋白功能缺陷，为临床诊断提供了分子依据。
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2. **文献名称**：*PEX1 Antibody Characterization in Peroxisome Biogenesis Disorders*
**作者**：Gärtner, J. et al.
**摘要**：该文献开发了一种高特异性PEX1抗体，用于检测过氧化物酶体生物合成障碍(PBDs)患者成纤维细胞中PEX1的亚细胞定位及稳定性，揭示了不同突变类型对蛋白稳定性的影响。
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3. **文献名称**：*Functional Analysis of PEX1 Variants Using Antibody-Based Assays*
**作者**：Ebberink, M.S. et al.
**摘要**：通过免疫印迹和免疫细胞化学技术，结合PEX1抗体评估了多种临床变异对PEX1蛋白功能的影响，建立了基于抗体检测的突变致病性分类方法。
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4. **文献名称**：*Structural Insights into PEX1-PEX6 Complex Dynamics via Epitope-Specific Antibodies*
**作者**：Miyata, N. & Fujiki, Y.
**摘要**：利用针对PEX1不同结构域的单克隆抗体，解析了PEX1与PEX6形成的ATP酶复合物在过氧化物酶体膜蛋白转运中的构象变化机制，为相关疾病的治疗提供了新靶点。
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**注**：以上文献信息为示例性内容，实际引用需根据具体数据库(如PubMed、Google Scholar)检索核实。
The PEX1 antibody is a crucial tool in studying peroxisome biogenesis disorders (PBDs), particularly Zellweger syndrome spectrum (ZSS). PEX1. a peroxin protein encoded by the PEX1 gene, is a member of the AAA ATPase family and plays a vital role in peroxisome assembly by facilitating protein import via membrane remodeling. Mutations in PEX1 are the most common cause of ZSS, characterized by defective peroxisomal functions, leading to severe metabolic, neurological, and developmental abnormalities.
PEX1 antibodies are primarily used in research to detect and quantify PEX1 protein expression in cellular or tissue samples, aiding in the diagnosis and mechanistic study of PBDs. They enable techniques like Western blotting, immunofluorescence, and immunohistochemistry to visualize PEX1 localization and assess its interaction with other peroxins (e.g., PEX6). These studies help elucidate how PEX1 mutations disrupt peroxisomal matrix protein import or cause protein mislocalization.
Commercial PEX1 antibodies are typically raised against specific epitopes of human PEX1. with validation in knockout controls to ensure specificity. Their applications extend to screening therapeutic strategies, such as chaperone drugs or gene therapy, aimed at rescuing PEX1 dysfunction. By advancing understanding of peroxisome biology and disease pathology, PEX1 antibodies remain indispensable in both basic research and clinical diagnostics for rare genetic disorders.
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