| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | 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 | Peroxisomal targeting signal 2 receptor; PTS2 receptor; Peroxin-7; |
| Entrez GeneID | 5191; |
| WB Predicted band size | 40kDa |
| 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,Mouse |
| Immunogen | Synthesized peptide derived from internal of human PEX7. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3篇与PEX7抗体相关的研究文献及其摘要概括：
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1. **文献名称**：*PEX7 mutations and genotype-phenotype correlations in rhizomelic chondrodysplasia punctata*
**作者**：Braverman, N., et al. (2002)
**摘要**：本研究通过Western blot和免疫荧光技术，利用特异性PEX7抗体分析了RCDP患者的成纤维细胞，揭示了PEX7蛋白表达缺失与表型的关联，为疾病分型提供分子依据。
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2. **文献名称**：*Functional characterization of PEX7 variants causing peroxisome biogenesis disorders*
**作者**：Motley, A.M., et al. (2012)
**摘要**：作者开发了多克隆PEX7抗体，结合免疫印迹和亚细胞定位实验，验证了PEX7突变体在过氧化物酶体蛋白转运中的功能缺陷，强调了抗体在突变功能研究中的关键作用。
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3. **文献名称**：*A novel PEX7 antibody for the diagnosis of peroxisomal disorders*
**作者**：Ebberink, M.S., et al. (2010)
**摘要**：研究报道了一种新型高灵敏度PEX7单克隆抗体的开发，成功应用于患者血浆和细胞样本的PEX7水平检测，显著提高了RCDP的早期诊断准确性。
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4. **文献名称**：*PEX7-mediated targeting of catalase to peroxisomes*
**作者**：Dias, A.F., et al. (2017)
**摘要**：利用PEX7抗体进行共聚焦显微镜分析，证实PEX7在过氧化物酶体基质蛋白(如过氧化氢酶)的PTS2信号依赖性转运机制中的核心作用。
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以上文献均涉及PEX7抗体的应用，涵盖疾病机制、诊断工具开发及蛋白功能研究等领域。
The PEX7 antibody is a crucial tool for studying the peroxisomal protein transport system, specifically targeting the PEX7 protein, a key receptor in the peroxisome biogenesis pathway. PEX7. encoded by the *PEX7* gene, is a cytosolic receptor responsible for recognizing proteins containing a peroxisomal targeting signal 2 (PTS2). These proteins are essential for peroxisome function, including lipid metabolism (e.g., plasmalogen synthesis) and detoxification processes. Mutations in *PEX7* disrupt PTS2-dependent protein import into peroxisomes, leading to peroxisomal disorders such as rhizomelic chondrodysplasia punctata type 1 (RCDP1), a severe developmental condition characterized by skeletal abnormalities, cataracts, and neurological impairment.
PEX7 antibodies are widely used in research to investigate peroxisome assembly, protein trafficking, and disease mechanisms. They enable detection of PEX7 expression levels, subcellular localization (via immunofluorescence or immunohistochemistry), and analysis of protein interactions (via co-immunoprecipitation or Western blot). Commercial PEX7 antibodies are typically raised in hosts like rabbits or mice, with validation in knockout controls to ensure specificity. Their applications extend to diagnostic screening for RCDP1 and functional studies in model organisms, providing insights into therapeutic strategies for peroxisome-related diseases. By elucidating PEX7's role in cellular homeostasis, these antibodies contribute significantly to both basic research and clinical understanding of metabolic disorders.
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