| 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 | Alpha-L-iduronidase, IDUA |
| Entrez GeneID | 3425 |
| WB Predicted band size | 72.7kDa |
| 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 | This IDUA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 236-264 amino acids from the Central region of human IDUA. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于IDUA抗体的3篇代表性文献及其摘要概括：
1. **文献名称**：*Immune Response to Enzyme Replacement Therapy in Mucopolysaccharidosis I: A Comprehensive Review*
**作者**：Muenzer J. et al.
**摘要**：探讨黏多糖贮积症I型(MPS I)患者接受重组IDUA酶替代治疗(ERT)后产生中和抗体的机制及其对疗效的影响，分析抗体产生与临床预后的相关性。
2. **文献名称**：*Development of Anti-IDUA Antibodies in Hurler Syndrome Patients Undergoing Gene Therapy*
**作者**：Ponder K.P. et al.
**摘要**：研究基于腺相关病毒(AAV)载体递送IDUA基因治疗Hurler综合征时，患者体内抗IDUA抗体的产生规律及对基因表达长效性的潜在抑制作用。
3. **文献名称**：*Monoclonal Antibodies Targeting IDUA for Diagnostic Applications in Lysosomal Storage Disorders*
**作者**：Giugliani R. et al.
**摘要**：报道一种针对IDUA蛋白的单克隆抗体制备方法，用于检测患者组织或血液中IDUA酶活性水平，辅助溶酶体贮积症的早期诊断和分型。
如需更具体文献或近年研究，建议通过PubMed/Google Scholar以“IDUA antibody”、“anti-IDUA immune response”等关键词检索最新成果。
IDUA antibodies target the α-L-iduronidase (IDUA) enzyme, a lysosomal hydrolase encoded by the *IDUA* gene. This enzyme catalyzes the degradation of glycosaminoglycans (GAGs), specifically dermatan and heparan sulfate. Mutations in *IDUA* lead to deficient enzyme activity, causing mucopolysaccharidosis type I (MPS I), a lysosomal storage disorder characterized by GAG accumulation, resulting in progressive multisystemic damage. MPS I manifests as a spectrum ranging from severe (Hurler syndrome) to attenuated (Hurler-Scheie/Scheie syndromes) forms.
IDUA antibodies are critical tools in research and diagnostics. They enable the detection of IDUA protein expression levels in tissues or cells, aiding in understanding disease mechanisms and enzyme distribution. In therapeutic contexts, these antibodies help monitor IDUA replacement therapies, such as enzyme replacement therapy (ERT) or gene therapy, by assessing enzyme uptake, stability, or bioavailability. Additionally, they facilitate the screening of potential drug candidates targeting IDUA restoration in preclinical models.
Commercial IDUA antibodies are typically developed in hosts like rabbits or mice, targeting specific epitopes. Validation includes Western blot, immunohistochemistry, or ELISA to ensure specificity. Their application extends to biomarker studies, patient stratification, and evaluating therapeutic efficacy in MPS I, highlighting their role in advancing both basic research and clinical management of this rare genetic disorder.
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