| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | IHC:1/100-1/200;IHF:1/50-1/200 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 1/20-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | PAH; PKU; PKU1;;PAH |
| WB Predicted band size | Calculated MW: 52 kDa ; Observed MW: 48 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 synthesized peptide derived from human PAH |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于PAH抗体的模拟参考文献示例(注：以下内容为虚构，仅用于演示格式)：
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1. **文献名称**: *Autoantibodies Targeting Phenylalanine Hydroxylase in Autoimmune Liver Diseases*
**作者**: Müller S, et al.
**摘要**: 研究在自身免疫性肝炎(AIH)患者血清中发现抗PAH抗体，并证实其与肝脏炎症严重程度呈正相关，提示其可能参与疾病进展。
2. **文献名称**: *PAH Antibody Detection in Phenylketonuria Patients Receiving Enzyme Therapy*
**作者**: Zhang Y, et al.
**摘要**: 通过酶联免疫吸附试验(ELISA)分析苯丙酮尿症(PKU)患者接受重组PAH替代治疗后的抗体产生，发现抗体水平与治疗耐药性相关。
3. **文献名称**: *Epitope Mapping of PAH Autoantibodies in Systemic Autoimmune Disorders*
**作者**: Tanaka K, et al.
**摘要**: 利用重组PAH蛋白片段鉴定了系统性红斑狼疮(SLE)患者中PAH抗体的主要抗原表位，为探索交叉免疫反应机制提供依据。
4. **文献名称**: *PAH Antibodies as a Novel Biomarker in Primary Biliary Cholangitis*
**作者**: Roberts E, et al.
**摘要**: 提出抗PAH抗体在原发性胆汁性胆管炎(PBC)中的特异性表达，并评估其作为早期诊断标志物的潜在价值。
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如需真实文献，建议通过PubMed或Google Scholar检索关键词“PAH antibody”“phenylalanine hydroxylase autoantibody”等获取最新研究。
**Background of PAH Antibodies**
Phenylalanine hydroxylase (PAH) is a hepatic enzyme critical for metabolizing phenylalanine into tyrosine, a process essential in preventing phenylketonuria (PKU), an autosomal recessive disorder characterized by phenylalanine accumulation. PAH dysfunction due to genetic mutations leads to PKU, necessitating lifelong dietary management. PAH antibodies typically arise in two contexts: autoimmune targeting of PAH or immune responses triggered by enzyme replacement therapies (ERT) or gene therapies.
In autoimmune contexts, anti-PAH antibodies are rare but implicated in immune-mediated liver dysfunction, though their clinical significance remains under investigation. More commonly, PAH antibodies are studied in therapeutic settings. Emerging treatments for PKU, such as pegylated recombinant PAH or gene therapy vectors, may provoke neutralizing antibodies, potentially reducing treatment efficacy. Monitoring anti-PAH antibody levels is thus crucial in clinical trials to assess immunogenicity and optimize dosing strategies.
Research also explores PAH antibodies as diagnostic tools. Immunoassays (e.g., ELISA, Western blot) detect PAH protein expression or mutations, aiding in PKU variant characterization. Current studies focus on modulating immune responses to PAH-targeted therapies, including tolerogenic protocols or engineered PAH variants with reduced immunogenicity. Understanding PAH antibody dynamics bridges translational gaps in PKU management and autoimmune hepatopathies.
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