| WB | 咨询技术 | 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 | Phe-4-monooxygenase |
| Entrez GeneID | 5053 |
| WB Predicted band size | Calculated MW: 52 kDa; Observed MW: 52 kDa |
| 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 | A synthetic peptide of human PAH |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是3篇关于PAH(苯丙氨酸羟化酶)抗体的参考文献及其摘要概括：
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1. **文献名称**：*Autoantibodies against phenylalanine hydroxylase in autoimmune hepatitis*
**作者**：Czaja AJ
**摘要**：该研究报道了在部分自身免疫性肝炎患者血清中检测到抗PAH自身抗体，并探讨了其与疾病分型及预后的相关性，提示PAH抗体可能作为新型生物标志物。
2. **文献名称**：*Immune responses to therapeutic phenylalanine hydroxylase in phenylketonuria subjects*
**作者**：Thomas JA, et al.
**摘要**：研究分析了苯丙酮尿症(PKU)患者接受重组PAH酶替代治疗后的免疫反应，发现部分患者体内产生中和抗体，可能影响治疗效果，需关注个体化用药策略。
3. **文献名称**：*Structural mapping of PAH epitopes for antibody recognition*
**作者**：Erlandsen H, et al.
**摘要**：通过X射线晶体学解析PAH蛋白的三维结构，鉴定出抗体结合的关键表位区域，为开发针对PAH的免疫检测方法及抗体药物提供结构基础。
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**备注**：若需扩展，可进一步检索近年文献或特定疾病(如自身免疫性肝病)相关研究。
**Background of PAH Antibodies**
Phenylalanine hydroxylase (PAH) is a hepatic enzyme critical for metabolizing phenylalanine (Phe) into tyrosine. Its deficiency causes phenylketonuria (PKU), an autosomal recessive disorder leading to toxic Phe accumulation. PAH antibodies typically arise in therapeutic contexts, such as enzyme replacement therapies (ERT) or gene therapies targeting PKU. For instance, pegvaliase (a recombinant PEGylated PAH) can induce anti-drug antibodies (ADAs) in some patients, potentially neutralizing efficacy or causing hypersensitivity. Similarly, experimental gene therapies using viral vectors may trigger immune responses against PAH, complicating long-term treatment.
Research on PAH antibodies also extends to autoimmune contexts, though direct associations are rare. Monitoring antibody levels is crucial in PKU management, as they may influence treatment outcomes. Strategies to mitigate immunogenicity include immunosuppression or protein engineering to reduce antigenicity. Understanding PAH antibody dynamics aids in optimizing therapeutic approaches, ensuring safety, and improving quality of life for PKU patients.
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