| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/2000-1/10000 | Human,Mouse,Rat |
| Aliases | COX6A; CMTRID; COX6AL |
| 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, Rat |
| Immunogen | Synthetic peptide of human COX6A1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下为3篇关于PPOX抗体的示例参考文献(注：以下为虚拟构造示例，非真实存在的文献)：
1. **《Development and validation of a PPOX antibody for porphyria diagnostics》**
- 作者：Smith J, et al.
- 摘要：研究开发了一种高特异性PPOX单克隆抗体，用于检测人类红细胞中原卟啉原氧化酶的活性缺陷，验证其在迟发性皮肤卟啉症(PCT)诊断中的敏感性和临床应用价值。
2. **《PPOX gene mutations and antibody-based detection in hereditary protoporphyria》**
- 作者：Lee S, et al.
- 摘要：通过免疫印迹和免疫荧光技术，分析PPOX基因突变患者的酶表达水平，证实抗体检测在遗传性原卟啉症分型中的重要性，并揭示特定突变对酶功能的影响。
3. **《Comparative study of PPOX antibody performance in immunohistochemistry》**
- 作者：Garcia R, et al.
- 摘要：比较不同商业来源的PPOX抗体在肝组织切片中的染色效果，提出优化实验条件以提高病理诊断中酶定位的准确性。
如需真实文献，建议通过PubMed、Web of Science或Google Scholar以关键词“PPOX antibody”“protoporphyrinogen oxidase diagnosis”检索近期研究。
PPOX antibodies target protoporphyrinogen oxidase (PPOX), a mitochondrial enzyme critical in the heme biosynthesis pathway. PPOX catalyzes the oxidation of protoporphyrinogen IX to protoporphyrin IX, the penultimate step before heme production. Defects in the PPOX gene are linked to variegate porphyria (VP), an autosomal dominant disorder characterized by skin photosensitivity and neurovisceral symptoms due to toxic porphyrin accumulation.
PPOX antibodies are primarily used in research and diagnostics to study enzyme expression, localization, and dysfunction. In VP, these antibodies help detect reduced PPOX activity or abnormal protein levels, aiding in confirming genetic diagnoses. They are also employed in mechanistic studies to explore how PPOX mutations disrupt heme synthesis and contribute to disease phenotypes.
Commercially available PPOX antibodies (polyclonal or monoclonal) are typically generated using recombinant human PPOX proteins or peptide fragments. Applications include Western blotting, immunohistochemistry, and immunofluorescence to assess tissue-specific expression patterns. Challenges include ensuring antibody specificity due to structural similarities among mitochondrial enzymes.
Overall, PPOX antibodies serve as vital tools for understanding porphyria pathophysiology, developing targeted therapies, and improving diagnostic accuracy for VP and related disorders.
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